Doff McElhinney

All Publications

• Targeted Multiplex Proteomics for the Development and Validation of Biomarkers in Primary Aldosteronism Subtyping. European journal of endocrinology Zhou, F., Ding, Y., Chen, T., Tang, Q., Zhang, J., Thyparambil, S., Jin, B., Han, Z., Chou, C. J., Schilling, J., Luo, R. Y., Tian, H., Sylvester, K. G., Whitin, J. C., Cohen, H. J., McElhinney, D. B., Tian, L., Ling, X. B., Ren, Y. 2024

  Abstract

  Primary aldosteronism (PA), a significant cause of secondary hypertension affecting approximately 10% of patients with severe hypertension, exacerbates cardiovascular and cerebrovascular complications even after blood pressure control. PA is categorized into two main subtypes: unilateral aldosterone-producing adenomas (APA) and bilateral hyperaldosteronism (BHA), each requiring distinct treatment approaches. Accurate subtype classification is crucial for selecting the most effective treatment. The goal of this study was to develop novel blood-based proteomic biomarkers to differentiate between APA and BHA subtypes in patients with PA.Five subtyping differential protein biomarker candidates (APOC3, CD56, CHGA, KRT5, and AZGP1) were identified through targeted proteomic profiling of plasma. The subtyping efficiency of these biomarkers was assessed at both the tissue gene expression and blood protein expression levels. To explore the underlying biology of APA and BHA, significant differential pathways were investigated.The five-protein panel proved highly effective in distinguishing APA from BHA in both tissue and blood samples. By integrating these five protein biomarkers with aldosterone and renin, our blood-based predictive methods achieved remarkable ROC AUCs of 0.986 (95% CI: 0.963-1.000) for differentiating essential hypertension (EH) from PA, and 0.922 (95% CI: 0.846-0.998) for subtyping APA versus BHA. These outcomes surpass the performance of the existing Kobayashi score subtyping system. Furthermore, the study validated differential pathways associated with the pathophysiology of primary aldosteronism, aligning with current scientific knowledge and opening new avenues for advancing PA care.The new blood-based biomarkers for PA subtyping hold the potential to significantly enhance clinical utility and advance the practice of PA care.

  View details for DOI 10.1093/ejendo/lvae148

  View details for PubMedID 39556467

• Don't Blame Balloon Atrial Septostomy! World journal for pediatric & congenital heart surgery McElhinney, D. B. 2024: 21501351241287129

  View details for DOI 10.1177/21501351241287129

  View details for PubMedID 39397576

• Durability of Aortic Homografts in Pulmonary Atresia and Major Aortopulmonary Collateral Arteries. World journal for pediatric & congenital heart surgery Ragheb, D. K., Martin, E., Zhang, Y., Jaggi, A., Asija, R., Peng, L. F., Ma, M., Hanley, F. L., McElhinney, D. B. 2024: 21501351241263752

  Abstract

  Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.

  View details for DOI 10.1177/21501351241263752

  View details for PubMedID 39166263

• Targeted multiplex validation of CSF proteomic biomarkers: implications for differentiation of PCNSL from tumor-free controls and other brain tumors. Frontiers in immunology Ma, J., Lin, Z., Zhang, Y., Ding, Y., Tang, Q., Qian, Y., Jin, B., Luo, R. Y., Liao, W. L., Thyparambil, S., Han, Z., Chou, C. J., Schilling, J., Li, Q., Zhang, M., Lin, Y., Ma, Y., Sylvester, K. G., Nagpal, S., McElhinney, D. B., Ling, X. B., Chen, B. 2024; 15: 1343109

  Abstract

  Primary central nervous system lymphoma (PCNSL) is a rare type of non-Hodgkin's lymphoma that affects brain parenchyma, eyes, cerebrospinal fluid, and spinal cord. Diagnosing PCNSL can be challenging because imaging studies often show similar patterns as other brain tumors, and stereotactic brain lesion biopsy conformation is invasive and not always possible. This study aimed to validate a previous proteomic profiling (PMID: 32610669) of cerebrospinal fluid (CSF) and develop a CSF-based proteomic panel for accurate PCNSL diagnosis and differentiation.CSF samples were collected from patients of 30 PCNSL, 30 other brain tumors, and 31 tumor-free/benign controls. Liquid chromatography tandem-mass spectrometry targeted proteomics analysis was used to establish CSF-based proteomic panels.Final proteomic panels were selected and optimized to diagnose PCNSL from tumor-free controls or other brain tumor lesions with an area under the curve (AUC) of 0.873 (95%CI: 0.723-0.948) and 0.937 (95%CI: 0.807- 0.985), respectively. Pathways analysis showed diagnosis panel features were significantly enriched in pathways related to extracellular matrices-receptor interaction, focal adhesion, and PI3K-Akt signaling, while prion disease, mineral absorption and HIF-1 signaling were significantly enriched with differentiation panel features.This study suggests an accurate clinical test panel for PCNSL diagnosis and differentiation with CSF-based proteomic signatures, which may help overcome the challenges of current diagnostic methods and improve patient outcomes.

  View details for DOI 10.3389/fimmu.2024.1343109

  View details for PubMedID 39144147

  View details for PubMedCentralID PMC11322575

• Transcatheter pulmonary valve replacement after arterial switch operation. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Nageotte, S., Salavitabar, A., Zablah, J. E., Ligon, R. A., Turner, M. E., El-Said, H., Guyon, P., Boucek, D., Alvarez-Fuente, M., McElhinney, D. B., Balzer, D., Shahanavaz, S. 2024

  Abstract

  Patients with d-transposition of the great arteries (d-TGA) who have undergone an arterial switch operation (ASO) can develop right ventricular outflow tract (RVOT) dysfunction with pulmonary regurgitation (PR) or stenosis. In these patients, treatment may include transcatheter pulmonary valve replacement (TPVR). Coronary compression is a contraindication occurring in 5% of typical TPVR cases. After ASO, there are various anatomical considerations that can confound TPVR, including potential coronary artery compression. Our goal is to understand feasibility of TPVR in patients following ASO.This was a retrospective multicenter cohort study of patients with RVOT dysfunction after ASO who underwent cardiac catheterization with intention to perform TPVR from 2008 to 2020.Across nine centers, 33 patients met inclusion criteria. TPVR was successful in 22 patients (66%), 19 receiving a Melody valve and 3 a SAPIEN valve. RVOT stenosis in isolation or with PR dictated need for TPVR in nearly all patients. One serious adverse event occurred with valve embolization. After TPVR, the RVOT peak gradient decreased from 43 to 9 mm Hg (p < 0.001); PR was trivial/none in all but one patient, in whom it was mild. Coronary compression prohibiting TPVR occurred in eight patients (24%) and two patients (6%) had severe aortic regurgitation from aortic root deformation precluding TPVR. Seven patients underwent RVOT reintervention with a median of 5.3 years post-TPVR.TPVR in patients with d-TGA after ASO is feasible, but in this cohort, coronary compression or aortic root distortion precluded TPVR in one-third of patients. The rate of RVOT reintervention after TPVR was higher in this cohort of ASO patients that in prior studies.

  View details for DOI 10.1002/ccd.31152

  View details for PubMedID 39033329

• Extravascular protrusion of the Alterra adaptive prestent identified on surveillance computed tomography imaging. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Gillespie, M. J., Maschietto, N., Aboulhosn, J. A., Balzer, D. T., Qureshi, A. M., McElhinney, D. B. 2024

  Abstract

  The Alterra adaptive prestent is a novel self-expanding device designed to provide a landing zone for the 29 mm SAPIEN 3 valve to treat pulmonary regurgitation in patients with a right ventricular outflow tract that is too large for a balloon expandable valve alone. The mechanism of fixation for the Alterra prestent is radial force from the self-expanding stent frame, combined with a unique set of flared "tines" that protrude from both ends of the stent.In this report, we describe 6 patients who underwent uncomplicated transcatheter pulmonary valve replacement with an Alterra adaptive prestent and SAPIEN 3 valve and had surveillance chest computed tomography (CT) scans performed 1 day to 21 months after implant. In each patient, the CT scan demonstrated extravascular extension of a portion of the Alterra prestent, without clinical sequelae, but with extension into the ascending aorta in 1 patient and contact with the ascending aorta, left pulmonary vein, or left atrial appendage in 3 others.Surveillance CT imaging shows that the Alterra prestent can perforate the pulmonary artery and/or right ventricle. Although no sequelae were seen in these patients, prestent perforation has the potential to be clinically important. Implanters should be aware of this finding and its potential implications. As experience with the Alterra prestent grows, it will be important to further define the risk factors, incidence, and implications of this phenomenon.

  View details for DOI 10.1002/ccd.31147

  View details for PubMedID 38967206

• Transcatheter Pulmonary Valve Replacement With the Harmony Valve in Patients Who Do Not Meet Recommended Oversizing Criteria on the Screening Perimeter Plot. Circulation. Cardiovascular interventions McElhinney, D. B., Gillespie, M. J., Aboulhosn, J. A., Cabalka, A. K., Morray, B. H., Balzer, D. T., Qureshi, A. M., Hoskoppal, A. K., Goldstein, B. H. 2024: e013889

  Abstract

  Anatomic selection for Harmony valve implant is determined with the aid of a screening report and perimeter plot (PP) that depicts the perimeter-derived radius along the right ventricular outflow tract (RVOT) and projects device oversizing. The PP provides an estimation of suitability for implant, but its sensitivity as a screening method is unknown. This study was performed to describe anatomic features and outcomes in patients who underwent Harmony TPV25 implant despite a PP that predicted inadequate oversizing.We reviewed RVOT anatomic features and measurements in patients who underwent transcatheter pulmonary valve replacement with the Harmony TPV25 device despite a PP that predicted inadequate oversizing.This study included 22 patients. There were no unsuccessful implants or adverse valve-related events. Anatomic features varied, but all patients fit into 1 of 3 anatomic types characterized by differences in RVOT dimensions. Type 1 patients (n=9) had a long RVOT with a choke point and a wide main pulmonary artery. Type 2 patients (n=6) had a short RVOT that was pyramidal in shape, with no choke point, and extensive main pulmonary artery lengthening/expansion during systole. Type 3 patients (n=7) had a short, bulbous main pulmonary artery with a choke point and an open pulmonary artery bifurcation.Transcatheter pulmonary valve replacement with the Harmony valve is feasible in some patients whose PP fit analysis predicts inadequate oversizing. All cases in this series fit into 1 of 3 anatomic patterns, which are not identified in the screening report. Implanters must review cases individually to assess the feasibility of the implant.

  View details for DOI 10.1161/CIRCINTERVENTIONS.123.013889

  View details for PubMedID 38606564

• Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry. Journal of the American College of Cardiology Goldstein, B. H., McElhinney, D. B., Gillespie, M. J., Aboulhosn, J. A., Levi, D. S., Morray, B. H., Cabalka, A. K., Love, B. A., Zampi, J. D., Balzer, D. T., Law, M. A., Schiff, M. D., Hoskoppal, A., Qureshi, A. M. 2024; 83 (14): 1310-1321

  Abstract

  Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021.In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience.This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S.The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention.A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2).In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes.

  View details for DOI 10.1016/j.jacc.2024.02.010

  View details for PubMedID 38569760

• Percutaneous Closure of a Ruptured Sinus of Valsalva Aneurysm under Transesophageal Echocardiography Guidance. CASE (Philadelphia, Pa.) Li, X. E., McElhinney, D. B., Lui, G. K., Clark, D. E., Woo, J. P. 2024; 8 (3Part A): 186-192

  Abstract

  • SOVA is a rare cardiac anomaly. • Ruptured SOVA carries a high mortality rate. • SOVA often coexists with other congenital lesions, most commonly VSD and bicuspid AV. • Ruptured SOVA needs repair; percutaneous repair is a safe alternative to surgery. • Echo plays a vital role in both diagnosing SOVA and guiding percutaneous closure.

  View details for DOI 10.1016/j.case.2023.12.006

  View details for PubMedID 38524988

  View details for PubMedCentralID PMC10954579

• Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals. Pediatric cardiology Long, Z. B., Punn, R., Zhang, Y., Arunamata, A. A., Asija, R., Ma, M., Hanley, F. L., McElhinney, D. B. 2024

  Abstract

  This study describes right ventricle (RV) characteristics and right ventricle to pulmonary artery (RV-PA) conduit function pre- and post-repair in patients with tetraology of Fallot with major aortopulmonary collaterals (TOF/MAPCAs). We reviewed patients who underwent single-stage, complete unifocalization, and repair of TOF/MAPCAs between 2006 and 2019 with available pre- and early postoperative echocardiograms. For a subset of patients, 6-12 month follow-up echocardiogram was available. RV and left ventricle (LV) characteristics and RV-PA conduit function were reviewed. Wilcoxon signed rank test and McNemar's test were used. 170 patients were reviewed, 46 had follow-up echocardiograms. Tricuspid valve annular plane systolic excursion (TAPSE) Z-scores were reduced from pre- (Z-score 0.01) to post-repair (Z-score -4.5, p < 0.001), improved but remained abnormal at follow-up (Z-score -4.0, p < 0.001). RV fractional area change (FAC) and LV ejection fraction were not significantly different before and after surgery. Conduit regurgitation was moderate or greater in 11% at discharge, increased to 65% at follow-up. RV-PA conduit failure (severe pulmonary stenosis or severe pulmonary regurgitation) was noted in 61, and 63% had dilated RV (diastolic RV area Z-score > 2) at follow-up. RV dilation correlated with the severe conduit regurgitation (p = 0.018). Longitudinal RV function was reduced after complete repair of TOF/MAPCAs, with decreased TAPSE and preserved FAC and LV ejection fraction. TAPSE improved but did not normalize at follow-up. Severe RV-PA conduit dysfunction was observed prior to discharge in 11% of patients and in 61% at follow-up. RV dilation was common at follow-up, especially in the presence of severe conduit regurgitation.

  View details for DOI 10.1007/s00246-024-03412-0

  View details for PubMedID 38360921

  View details for PubMedCentralID 6035101

• Non-invasive Estimation of Pressure Drop Across Aortic Coarctations: Validation of 0D and 3D Computational Models with In Vivo Measurements. Annals of biomedical engineering Nair, P. J., Pfaller, M. R., Dual, S. A., McElhinney, D. B., Ennis, D. B., Marsden, A. L. 2024

  Abstract

  Blood pressure gradient ([Formula: see text]) across an aortic coarctation (CoA) is an important measurement to diagnose CoA severity and gauge treatment efficacy. Invasive cardiac catheterization is currently the gold-standard method for measuring blood pressure. The objective of this study was to evaluate the accuracy of [Formula: see text] estimates derived non-invasively using patient-specific 0D and 3D deformable wall simulations. Medical imaging and routine clinical measurements were used to create patient-specific models of patients with CoA (N = 17). 0D simulations were performed first and used to tune boundary conditions and initialize 3D simulations. [Formula: see text] across the CoA estimated using both 0D and 3D simulations were compared to invasive catheter-based pressure measurements for validation. The 0D simulations were extremely efficient ([Formula: see text] 15 s computation time) compared to 3D simulations ([Formula: see text] 30 h computation time on a cluster). However, the 0D [Formula: see text] estimates, unsurprisingly, had larger mean errors when compared to catheterization than 3D estimates (12.1 ± 9.9 mmHg vs 5.3 ± 5.4 mmHg). In particular, the 0D model performance degraded in cases where the CoA was adjacent to a bifurcation. The 0D model classified patients with severe CoA requiring intervention (defined as [Formula: see text] [Formula: see text] 20 mmHg) with 76% accuracy and 3D simulations improved this to 88%. Overall, a combined approach, using 0D models to efficiently tune and launch 3D models, offers the best combination of speed and accuracy for non-invasive classification of CoA severity.

  View details for DOI 10.1007/s10439-024-03457-5

  View details for PubMedID 38341399

• Global metabolomics revealed deviations from the metabolic aging clock in colorectal cancer patients. Theranostics Zhang, L., Mo, S., Zhu, X., Chou, C. J., Jin, B., Han, Z., Schilling, J., Liao, W., Thyparambil, S., Luo, R. Y., Whitin, J. C., Tian, L., Nagpal, S., Ceresnak, S. R., Cohen, H. J., McElhinney, D. B., Sylvester, K. G., Gong, Y., Fu, C., Ling, X. B., Peng, J. 2024; 14 (4): 1602-1614

  Abstract

  Background: Markers of aging hold promise in the context of colorectal cancer (CRC) care. Utilizing high-resolution metabolomic profiling, we can unveil distinctive age-related patterns that have the potential to predict early CRC development. Our study aims to unearth a panel of aging markers and delve into the metabolomic alterations associated with aging and CRC. Methods: We assembled a serum cohort comprising 5,649 individuals, consisting of 3,002 healthy volunteers, 715 patients diagnosed with colorectal advanced precancerous lesions (APL), and 1,932 CRC patients, to perform a comprehensive metabolomic analysis. Results: We successfully identified unique age-associated patterns across 42 metabolic pathways. Moreover, we established a metabolic aging clock, comprising 9 key metabolites, using an elastic net regularized regression model that accurately estimates chronological age. Notably, we observed significant chronological disparities among the healthy population, APL patients, and CRC patients. By combining the analysis of circulative carcinoembryonic antigen levels with the categorization of individuals into the "hypo" metabolic aging subgroup, our blood test demonstrates the ability to detect APL and CRC with positive predictive values of 68.4% (64.3%, 72.2%) and 21.4% (17.8%, 25.9%), respectively. Conclusions: This innovative approach utilizing our metabolic aging clock holds significant promise for accurately assessing biological age and enhancing our capacity to detect APL and CRC.

  View details for DOI 10.7150/thno.87303

  View details for PubMedID 38389840

  View details for PubMedCentralID PMC10879879

• Transcatheter Pulmonary Valve Replacement With Balloon-Expandable Valves: Utilization and Procedural Outcomes From the IMPACT Registry. JACC. Cardiovascular interventions Stefanescu Schmidt, A. C., Armstrong, A. K., Aboulhosn, J. A., Kennedy, K. F., Jones, T. K., Levi, D. S., McElhinney, D. B., Bhatt, A. B. 2024; 17 (2): 231-244

  Abstract

  Transcatheter pulmonary valve replacement (TPVR) has expanded and evolved since its initial commercial approval in the United States in 2010.This study sought to characterize real-world practice, including patient selection, procedural outcomes, complications, and off-label usage.Characteristics and outcomes for patients undergoing balloon-expandable TPVR were collected from the American College of Cardiology National Cardiovascular Data Registry IMPACT (Improving Pediatric and Adult Congenital Treatment) Registry.Between April 2016 and March 2021, 4,513 TPVR procedures were performed in patients with a median age of 19 years, 57% with a Melody (Medtronic Inc) and 43% with a SAPIEN (Edwards Lifesciences) valve. Most implanting centers performed <10 cases annually. One-third of transcatheter pulmonary valve implants were into homograft conduits, one-third were into bioprosthetic valves (BPVs), 25% were in native or patched right ventricular outflow tracts (RVOTs), and 6% were into Contegra (Medtronic Inc) conduits. Over the course of the study period, SAPIEN valve use grew from ∼25% to 60%, in large part because of implants in patients with a native/patched RVOT. Acute success was achieved in 95% of patients (95.7% in homografts, 96.2% in BPVs, 94.2% in native RVOTs, and 95.4% in Contegra conduits). Major adverse events occurred in 2.4% of procedures, more commonly in patients with a homograft (2.9%) or native RVOT (3.4%) than a prior BPV (1.4%; P = 0.004).This study describes novel population data on the use and procedural outcomes of TPVR with balloon-expandable valves. Over time, there has been increasing use of TPVR to treat regurgitant native RVOT anatomy, with the SAPIEN valve more commonly used for this application.

  View details for DOI 10.1016/j.jcin.2023.10.065

  View details for PubMedID 38267137

• Multicenter Study of Survival Benefit of Cardiac Resynchronization Therapy in Pediatric and Congenital Heart Disease. JACC. Clinical electrophysiology Chubb, H., Mah, D. Y., Shah, M., Lin, K. Y., Peng, D. M., Hale, B. W., May, L., Etheridge, S., Goodyer, W., Ceresnak, S. R., Motonaga, K. S., Rosenthal, D. N., Almond, C. S., McElhinney, D. B., Dubin, A. M. 2023

  Abstract

  BACKGROUND: Evidence for the efficacy of cardiac resynchronization therapy (CRT) in pediatric and congenital heart disease (CHD) has been limited to surrogate outcomes.OBJECTIVES: This study aimed to assess the impact of CRT upon the risk of transplantation or death in a retrospective, high-risk, controlled cohort at 5 quaternary referral centers.METHODS: Both CRT patients and control patients were<21 years of age or had CHD; had systemic ventricular ejection fraction<45%; symptomatic heart failure; and significant electrical dyssynchrony (QRS duration z score >3 or single-site ventricular pacing >40%) at enrollment. Patients with CRT were matched with control patients via 1:1 propensity score matching. CRT patients were enrolled at CRT implantation; control patients were enrolled at the outpatient clinical encounter where inclusion criteria were first met. The primary endpoint was transplantation or death.RESULTS: In total, 324 control patients and 167 CRT recipients were identified. Mean follow-up was 4.2 ± 3.7 years. Upon propensity score matching, 139 closely matched pairs were identified (20 baseline indices). Of the 139 matched pairs, 52 (37.0%) control patients and 31 (22.0%) CRT recipients reached the primary endpoint. On both unadjusted and multivariable Cox regression analysis, the risk reduction associated with CRT for the primary endpoint was significant (HR: 0.40; 95%CI: 0.25-0.64; P< 0.001; and HR: 0.44; 95%CI: 0.28-0.71; P=0.001, respectively). On longitudinal assessment, the CRT group had significantly improved systemic ventricular ejection fraction (P< 0.001) and shorter QRS duration (P=0.015), sustained to 5 years.CONCLUSIONS: In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplantation-free survival.

  View details for DOI 10.1016/j.jacep.2023.11.008

  View details for PubMedID 38206260

• Correction: Exploring the feasibility of using long-term stored newborn dried blood spots to identify metabolic features for congenital heart disease screening. Biomarker research Ceresnak, S. R., Zhang, Y., Ling, X. B., Su, K. J., Tang, Q., Jin, B., Schilling, J., Chou, C. J., Han, Z., Floyd, B. J., Whitin, J. C., Hwa, K. Y., Sylvester, K. G., Chubb, H., Luo, R. Y., Tian, L., Cohen, H. J., McElhinney, D. B. 2023; 11 (1): 101

  View details for DOI 10.1186/s40364-023-00546-w

  View details for PubMedID 37993911

• Retrieval of Large Balloon Fragments During Transcatheter Pulmonary ValveImplantation Using a Novel Retrieval System. JACC. Case reports Taylor, A. C., Ghandour, M. A., Khan, A., Gowda, S. T., Nunez-Gallegos, F., Peng, L. F., Aboulhosn, J. A., Levi, D. S., McElhinney, D. B., Qureshi, A. M. 2023; 26: 102058

  Abstract

  The removal of balloon fragments from the pulmonary artery without damaging the pulmonary and tricuspid valves can be difficult. Four cases during transcatheter pulmonary valve replacement are described in which a novel retrieval system was used to facilitate safe removal. (Level of Difficulty: Advanced.).

  View details for DOI 10.1016/j.jaccas.2023.102058

  View details for PubMedID 38094173

• Exploring the feasibility of using long-term stored newborn dried blood spots to identify metabolic features for congenital heart disease screening. Biomarker research Ceresnak, S. R., Zhang, Y., Ling, X. B., Su, K. J., Tang, Q., Jin, B., Schilling, J., Chou, C. J., Han, Z., Floyd, B. J., Whitin, J. C., Hwa, K. Y., Sylvester, K. G., Chubb, H., Luo, R. Y., Tian, L., Cohen, H. J., McElhinney, D. B. 2023; 11 (1): 97

  Abstract

  Congenital heart disease (CHD) represents a significant contributor to both morbidity and mortality in neonates and children. There's currently no analogous dried blood spot (DBS) screening for CHD immediately after birth. This study was set to assess the feasibility of using DBS to identify reliable metabolite biomarkers with clinical relevance, with the aim to screen and classify CHD utilizing the DBS. We assembled a cohort of DBS datasets from the California Department of Public Health (CDPH) Biobank, encompassing both normal controls and three pre-defined CHD categories. A DBS-based quantitative metabolomics method was developed using liquid chromatography with tandem mass spectrometry (LC-MS/MS). We conducted a correlation analysis comparing the absolute quantitated metabolite concentration in DBS against the CDPH NBS records to verify the reliability of metabolic profiling. For hydrophilic and hydrophobic metabolites, we executed significant pathway and metabolite analyses respectively. Logistic and LightGBM models were established to aid in CHD discrimination and classification. Consistent and reliable quantification of metabolites were demonstrated in DBS samples stored for up to 15 years. We discerned dysregulated metabolic pathways in CHD patients, including deviations in lipid and energy metabolism, as well as oxidative stress pathways. Furthermore, we identified three metabolites and twelve metabolites as potential biomarkers for CHD assessment and subtypes classifying. This study is the first to confirm the feasibility of validating metabolite profiling results using long-term stored DBS samples. Our findings highlight the potential clinical applications of our DBS-based methods for CHD screening and subtype classification.

  View details for DOI 10.1186/s40364-023-00536-y

  View details for PubMedID 37957758

  View details for PubMedCentralID PMC10644604

• Right ventricular outflow tract obstruction associated with neointimal tissue accumulation and distortion of the Harmony TPV25 stent frame: Potential mechanisms and treatment. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Steinberg, Z. L., Cabalka, A. K., Balzer, D. T., Asnes, J. D., Morray, B. H., Gillespie, M. J., McElhinney, D. B. 2023

  Abstract

  The Harmony TPV25 transcatheter pulmonary valve (Medtronic Inc.) is constructed with a self-expanding stent frame comprising six zigged nitinol wires sewn together and covered with knitted polyester fabric, with flared inflow and outflow ends and a porcine pericardial valve sutured to the central portion of the device. It was approved for treatment of pulmonary regurgitation after prior right ventricular outflow tract repair in 2021. Early outcomes of this procedure have been excellent, but little is known about valve durability or ultimate mechanisms of dysfunction.We collected data on patients who underwent reintervention for TPV25 dysfunction and described findings related to distortion of the stent frame and tissue accumulation.We describe six patients who underwent valve-in-valve implant for TPV25 obstruction (peak catheterization gradient peak 28-73 mmHg) 10-28 months after implant. In all cases, there was tissue accumulation within the inflow and valve-housing segments of the device and deformation of the self-expanding valve frame characterized by variable circumferential narrowing at the junction between the valve housing and the inflow and outflow portions of the device, with additional geometric changes in all segments. All six patients underwent valve-in-valve implant that results in a final peak gradient ≤10 mmHg and no regurgitation.The occurrence of short-term Harmony TPV25 dysfunction in multiple patients with a similar appearance of frame distortion and tissue accumulation within the inflow and valve housing portions of the device suggests that this may be an important failure mechanism for this valve. Potential causes of the observed findings are discussed. It is possible to treat this mechanism of TPV25 dysfunction with valve-in-valve implant using balloon expandable transcatheter valves.

  View details for DOI 10.1002/ccd.30901

  View details for PubMedID 37925624

• High-throughput quantitation of amino acids and acylcarnitine in cerebrospinal fluid: identification of PCNSL biomarkers and potential metabolic messengers FRONTIERS IN MOLECULAR BIOSCIENCES Ma, J., Chen, K., Ding, Y., Li, X., Tang, Q., Jin, B., Luo, R. Y., Thyparambil, S., Han, Z., Chou, C., Zhou, A., Schilling, J., Lin, Z., Ma, Y., Li, Q., Zhang, M., Sylvester, K. G., Nagpal, S., McElhinney, D. B., Ling, X. B., Chen, B. 2023; 10

  View details for DOI 10.3389/fmolb.2023.1257079

  View details for Web of Science ID 001101640200001

• High-throughput quantitation of amino acids and acylcarnitine in cerebrospinal fluid: identification of PCNSL biomarkers and potential metabolic messengers. Frontiers in molecular biosciences Ma, J., Chen, K., Ding, Y., Li, X., Tang, Q., Jin, B., Luo, R. Y., Thyparambil, S., Han, Z., Chou, C. J., Zhou, A., Schilling, J., Lin, Z., Ma, Y., Li, Q., Zhang, M., Sylvester, K. G., Nagpal, S., McElhinney, D. B., Ling, X. B., Chen, B. 2023; 10: 1257079

  Abstract

  Background: Due to the poor prognosis and rising occurrence, there is a crucial need to improve the diagnosis of Primary Central Nervous System Lymphoma (PCNSL), which is a rare type of non-Hodgkin's lymphoma. This study utilized targeted metabolomics of cerebrospinal fluid (CSF) to identify biomarker panels for the improved diagnosis or differential diagnosis of primary central nervous system lymphoma (PCNSL). Methods: In this study, a cohort of 68 individuals, including patients with primary central nervous system lymphoma (PCNSL), non-malignant disease controls, and patients with other brain tumors, was recruited. Their cerebrospinal fluid samples were analyzed using the Ultra-high performance liquid chromatography - tandem mass spectrometer (UHPLC-MS/MS) technique for targeted metabolomics analysis. Multivariate statistical analysis and logistic regression modeling were employed to identify biomarkers for both diagnosis (Dx) and differential diagnosis (Diff) purposes. The Dx and Diff models were further validated using a separate cohort of 34 subjects through logistic regression modeling. Results: A targeted analysis of 45 metabolites was conducted using UHPLC-MS/MS on cerebrospinal fluid (CSF) samples from a cohort of 68 individuals, including PCNSL patients, non-malignant disease controls, and patients with other brain tumors. Five metabolic features were identified as biomarkers for PCNSL diagnosis, while nine metabolic features were found to be biomarkers for differential diagnosis. Logistic regression modeling was employed to validate the Dx and Diff models using an independent cohort of 34 subjects. The logistic model demonstrated excellent performance, with an AUC of 0.83 for PCNSL vs. non-malignant disease controls and 0.86 for PCNSL vs. other brain tumor patients. Conclusion: Our study has successfully developed two logistic regression models utilizing metabolic markers in cerebrospinal fluid (CSF) for the diagnosis and differential diagnosis of PCNSL. These models provide valuable insights and hold promise for the future development of a non-invasive and reliable diagnostic tool for PCNSL.

  View details for DOI 10.3389/fmolb.2023.1257079

  View details for PubMedID 38028545

  View details for PubMedCentralID PMC10644155

• Fontan Associated Liver Disease: Canary in the Coal Mine or Silent Killer? Journal of the American Heart Association Haeffele, C., McElhinney, D. B. 2023: e031597

  View details for DOI 10.1161/JAHA.123.031597

  View details for PubMedID 37776191

• Recommendations for centers performing pediatric heart surgery in the United States. The Journal of thoracic and cardiovascular surgery Backer, C. L., Overman, D. M., Dearani, J. A., Romano, J. C., Tweddell, J. S., Kumar, S. R., Marino, B. S., Bacha, E. A., Jaquiss, R. D., Zaidi, A. N., Gurvitz, M., Costello, J. M., Pierick, T. A., Ravekes, W. J., Reagor, J. A., St Louis, J. D., Spaeth, J., Mahle, W. T., Shin, A. Y., Lopez, K. N., Karamlou, T., Welke, K. F., Bryant, R., Husain, S. A., Chen, J. M., Kaza, A., Wells, W. J., Glatz, A. C., Cohen, M. I., McElhinney, D. B., Parra, D. A., Pasquali, S. K. 2023

  Abstract

  Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

  View details for DOI 10.1016/j.jtcvs.2023.09.001

  View details for PubMedID 37777958

• Recommendations for Centers Performing Pediatric Heart Surgery in the United States. The Annals of thoracic surgery Backer, C. L., Overman, D. M., Dearani, J. A., Romano, J. C., Tweddell, J. S., Kumar, S. R., Marino, B. S., Bacha, E. A., Jaquiss, R. D., Zaidi, A. N., Gurvitz, M., Costello, J. M., Pierick, T. A., Ravekes, W. J., Reagor, J. A., St Louis, J. D., Spaeth, J., Mahle, W. T., Shin, A. Y., Lopez, K. N., Karamlou, T., Welke, K. F., Bryant, R., Husain, S. A., Chen, J. M., Kaza, A., Wells, W. J., Glatz, A. C., Cohen, M. I., McElhinney, D. B., Parra, D. A., Pasquali, S. K. 2023

  Abstract

  Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

  View details for DOI 10.1016/j.athoracsur.2023.08.016

  View details for PubMedID 37777933

• 20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries. Journal of the American College of Cardiology McElhinney, D. B., Asija, R., Zhang, Y., Jaggi, A., Shek, J., Peng, L. F., Boltz, M. G., Ma, M., Martin, E., Hanley, F. L. 2023; 82 (12): 1206-1222

  Abstract

  BACKGROUND: We have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments.OBJECTIVES: The purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs.METHODS: We reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December2021.RESULTS: During the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P=0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95%CI: 1.4-5.7; P=0.004), preoperative respiratory support (HR: 2.0; 95%CI: 1.2-3.3; P=0.008), and palliative first surgery at our center (HR: 3.5; 95%CI: 2.3-5.4; P< 0.001) were associated with higher risk of death.CONCLUSIONS: In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis andMAPCAs have continued to improve.

  View details for DOI 10.1016/j.jacc.2023.06.041

  View details for PubMedID 37704311

• Altered expression of the L-arginine/nitric oxide pathway in ovarian cancer: metabolic biomarkers and biological implications. BMC cancer Chen, L., Tang, Q., Zhang, K., Huang, Q., Ding, Y., Jin, B., Liu, S., Hwa, K., Chou, C. J., Zhang, Y., Thyparambil, S., Liao, W., Han, Z., Mortensen, R., Schilling, J., Li, Z., Heaton, R., Tian, L., Cohen, H. J., Sylvester, K. G., Arent, R. C., Zhao, X., McElhinney, D. B., Wu, Y., Bai, W., Ling, X. B. 2023; 23 (1): 844

  Abstract

  Ovarian cancer (OC) is a highly lethal gynecological malignancy. Extensive research has shown that OC cells undergo significant metabolic alterations during tumorigenesis. In this study, we aim to leverage these metabolic changes as potential biomarkers for assessing ovarian cancer.A functional module-based approach was utilized to identify key gene expression pathways that distinguish different stages of ovarian cancer (OC) within a tissue biopsy cohort. This cohort consisted of control samples (n = 79), stage I/II samples (n = 280), and stage III/IV samples (n = 1016). To further explore these altered molecular pathways, minimal spanning tree (MST) analysis was applied, leading to the formulation of metabolic biomarker hypotheses for OC liquid biopsy. To validate, a multiple reaction monitoring (MRM) based quantitative LCMS/MS method was developed. This method allowed for the precise quantification of targeted metabolite biomarkers using an OC blood cohort comprising control samples (n = 464), benign samples (n = 3), and OC samples (n = 13).Eleven functional modules were identified as significant differentiators (false discovery rate, FDR < 0.05) between normal and early-stage, or early-stage and late-stage ovarian cancer (OC) tumor tissues. MST analysis revealed that the metabolic L-arginine/nitric oxide (L-ARG/NO) pathway was reprogrammed, and the modules related to "DNA replication" and "DNA repair and recombination" served as anchor modules connecting the other nine modules. Based on this analysis, symmetric dimethylarginine (SDMA) and arginine were proposed as potential liquid biopsy biomarkers for OC assessment. Our quantitative LCMS/MS analysis on our OC blood cohort provided direct evidence supporting the use of the SDMA-to-arginine ratio as a liquid biopsy panel to distinguish between normal and OC samples, with an area under the ROC curve (AUC) of 98.3%.Our comprehensive analysis of tissue genomics and blood quantitative LC/MSMS metabolic data shed light on the metabolic reprogramming underlying OC pathophysiology. These findings offer new insights into the potential diagnostic utility of the SDMA-to-arginine ratio for OC assessment. Further validation studies using adequately powered OC cohorts are warranted to fully establish the clinical effectiveness of this diagnostic test.

  View details for DOI 10.1186/s12885-023-11192-8

  View details for PubMedID 37684587

  View details for PubMedCentralID 8192829

• Non-invasive estimation of pressure drop across aortic coarctations: validation of 0D and 3D computational models with in vivo measurements. medRxiv : the preprint server for health sciences Nair, P. J., Pfaller, M. R., Dual, S. A., McElhinney, D. B., Ennis, D. B., Marsden, A. L. 2023

  Abstract

  Blood pressure gradient (ΔP) across an aortic coarctation (CoA) is an important measurement to diagnose CoA severity and gauge treatment efficacy. Invasive cardiac catheterization is currently the gold-standard method for measuring blood pressure. The objective of this study was to evaluate the accuracy of ΔP estimates derived non-invasively using patient-specific 0D and 3D deformable wall simulations.Medical imaging and routine clinical measurements were used to create patient-specific models of patients with CoA (N=17). 0D simulations were performed first and used to tune boundary conditions and initialize 3D simulations. ΔP across the CoA estimated using both 0D and 3D simulations were compared to invasive catheter-based pressure measurements for validation.The 0D simulations were extremely efficient (~15 secs computation time) compared to 3D simulations (~30 hrs computation time on a cluster). However, the 0D ΔP estimates, unsurprisingly, had larger mean errors when compared to catheterization than 3D estimates (12.1 ± 9.9 mmHg vs 5.3 ± 5.4 mmHg). In particular, the 0D model performance degraded in cases where the CoA was adjacent to a bifurcation. The 0D model classified patients with severe CoA requiring intervention (defined as ΔP≥20 mmHg) with 76% accuracy and 3D simulations improved this to 88%.Overall, a combined approach, using 0D models to efficiently tune and launch 3D models, offers the best combination of speed and accuracy for non-invasive classification of CoA severity.

  View details for DOI 10.1101/2023.09.05.23295066

  View details for PubMedID 37732242

  View details for PubMedCentralID PMC10508787

• Recommendations for Centers Performing Pediatric Heart Surgery in the United States. World journal for pediatric & congenital heart surgery Backer, C. L., Overman, D. M., Dearani, J. A., Romano, J. C., Tweddell, J. S., Ram Kumar, S., Marino, B. S., Bacha, E. A., Jaquiss, R. D., Zaidi, A. N., Gurvitz, M., Costello, J. M., Pierick, T. A., Ravekes, W. J., Reagor, J. A., St Louis, J. D., Spaeth, J., Mahle, W. T., Shin, A. Y., Lopez, K. N., Karamlou, T., Welke, K. F., Bryant, R., Adil Husain, S., Chen, J. M., Kaza, A., Wells, W. J., Glatz, A. C., Cohen, M. I., McElhinney, D. B., Parra, D. A., Pasquali, S. K. 2023; 14 (5): 642-679

  Abstract

  Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

  View details for DOI 10.1177/21501351231190353

  View details for PubMedID 37737602

• Short- and Mid-Term Results of Pulmonary Valve Replacement with the Inspiris Valve. The Annals of thoracic surgery Ragheb, D. K., Martin, E., Jaggi, A., Lui, G. K., Maskatia, S. A., Ma, M., Hanley, F. L., McElhinney, D. B. 2023

  Abstract

  BACKGROUND: Various bioprosthetic valves are used off-label for pulmonary valve replacement (PVR), but there is no consensus about whether a particular valve is best for this application. Recently, the Inspiris Valve was approved for aortic valve replacement, and surgeons have begun utilizing it for PVR. There is limited evidence about the performance of the Inspiris valve compared to other valves in the pulmonary position.METHODS: We reviewed all patients who underwent PVR with a size 19-27mm Inspiris or Mosaic valve from 2007-2022. Mid-term outcomes included freedom from moderate or severe pulmonary regurgitation (PR), a maximum Doppler gradient >36mmHg, and freedom from reintervention.RESULTS: A total of 225 consecutive patients who underwent PVR with a size 19-27mm Mosaic (n=163) or Inspiris (n=62) valve were included. There was no difference in baseline characteristics. Early postoperative gradients were low in both groups, but higher in the Mosaic cohort, and neither group had more than mild PR on discharge. On uni- and multivariable analysis, Inspiris valves were significantly more likely to develop moderate or greater PR over time. There was no significant difference between the valves in freedom from reintervention or from a maximum gradient >36mmHg.CONCLUSIONS: Early and short-term gradients were similar in patients undergoing PVR with Inspiris and Mosaic valves, but significant PR was more common in patients who received an Inspiris valve. These preliminary findings suggest that the durability of the Inspiris valve in the pulmonary position may not be superior to other bioprosthetic valves used for PVR.

  View details for DOI 10.1016/j.athoracsur.2023.07.049

  View details for PubMedID 37625611

• Evolving Utilization of Covered Stents for Treatment of Aortic Coarctation: Report From the IMPACT Registry. Circulation. Cardiovascular interventions Morray, B. H., Kennedy, K. F., McElhinney, D. B. 2023: e012697

  Abstract

  BACKGROUND: Covered stent (CS) use for the transcatheter treatment of coarctation of the aorta (COA) was first described over 20 years ago. The covered Cheatham-platinum stent received Food and Drug Administration approval for COA treatment in 2016. Data collected as part of the National Cardiovascular Data Registry IMPACT registry were evaluated for contemporary usage patterns of CS for the treatment of COA from 2016 to 2021.METHODS: Version 2 of the IMPACT registry was queried for all patients who underwent stent placement for treatment of COA from 2016 to 2021. Trends in CS use were evaluated by year and age of patient at the time of implant. Analysis was limited to clinical factors collected as part of the registry and was performed to identify factors associated with CS use.RESULTS: Data were available for 1989 case entries. Most patients (92%) received a single stent. The overall percentage of CS use in the cohort was 23% and remained consistent throughout the study period. The probability of CS use was significantly associated with increasing patient age at implant. Additional factors associated with CS use included smaller initial COA diameter, native COA, and the presence of a pseudoaneurysm. Procedural adverse event rates were low.CONCLUSIONS: CS use to treat COA was more common in adult patients and remained stable over the study period. Factors associated with CS use, including smaller COA diameter and aortic pseudoaneurysm, demonstrate the perceived value of CS as a tool to reduce the risk of aortic wall injury during treatment of COA.

  View details for DOI 10.1161/CIRCINTERVENTIONS.122.012697

  View details for PubMedID 37417230

• Comparison of Immersed Boundary Simulations of Heart Valve Hemodynamics Against In Vitro 4D Flow MRI Data. Annals of biomedical engineering Kaiser, A. D., Schiavone, N. K., Elkins, C. J., McElhinney, D. B., Eaton, J. K., Marsden, A. L. 2023

  Abstract

  The immersed boundary (IB) method is a mathematical framework for fluid-structure interaction problems (FSI) that was originally developed to simulate flows around heart valves. Direct comparison of FSI simulations around heart valves against experimental data is challenging, however, due to the difficulty of performing robust and effective simulations, the complications of modeling a specific physical experiment, and the need to acquire experimental data that is directly comparable to simulation data. Such comparators are a necessary precursor for further formal validation studies of FSI simulations involving heart valves. In this work, we performed physical experiments of flow through a pulmonary valve in an in vitro pulse duplicator, and measured the corresponding velocity field using 4D flow MRI (4-dimensional flow magnetic resonance imaging). We constructed a computer model of this pulmonary artery setup, including modeling valve geometry and material properties via a technique called design-based elasticity, and simulated flow through it with the IB method. The simulated flow fields showed excellent qualitative agreement with experiments, excellent agreement on integral metrics, and reasonable relative error in the entire flow domain and on slices of interest. These results illustrate how to construct a computational model of a physical experiment for use as a comparator.

  View details for DOI 10.1007/s10439-023-03266-2

  View details for PubMedID 37378877

  View details for PubMedCentralID 6328065

• Development of a Urine Metabolomics Biomarker-Based Prediction Model for Preeclampsia during Early Pregnancy. Metabolites Zhang, Y., Sylvester, K. G., Jin, B., Wong, R. J., Schilling, J., Chou, C. J., Han, Z., Luo, R. Y., Tian, L., Ladella, S., Mo, L., Maric, I., Blumenfeld, Y. J., Darmstadt, G. L., Shaw, G. M., Stevenson, D. K., Whitin, J. C., Cohen, H. J., McElhinney, D. B., Ling, X. B. 2023; 13 (6)

  Abstract

  Preeclampsia (PE) is a condition that poses a significant risk of maternal mortality and multiple organ failure during pregnancy. Early prediction of PE can enable timely surveillance and interventions, such as low-dose aspirin administration. In this study, conducted at Stanford Health Care, we examined a cohort of 60 pregnant women and collected 478 urine samples between gestational weeks 8 and 20 for comprehensive metabolomic profiling. By employing liquid chromatography mass spectrometry (LCMS/MS), we identified the structures of seven out of 26 metabolomics biomarkers detected. Utilizing the XGBoost algorithm, we developed a predictive model based on these seven metabolomics biomarkers to identify individuals at risk of developing PE. The performance of the model was evaluated using 10-fold cross-validation, yielding an area under the receiver operating characteristic curve of 0.856. Our findings suggest that measuring urinary metabolomics biomarkers offers a noninvasive approach to assess the risk of PE prior to its onset.

  View details for DOI 10.3390/metabo13060715

  View details for PubMedID 37367874

• Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation. Circulation. Arrhythmia and electrophysiology Giacone, H. M., Chubb, H., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R., Goodyer, W. R., Hanish, D., Trela, A. V., Boramanand, N., Lencioni, E., Boothroyd, D., Graber-Naidich, A., Wright, G., Haeffele, C., Hollander, S. A., McElhinney, D. B., Ma, M., Hanley, F. L., Chen, S. 2023: e011143

  Abstract

  With the advent of more intensive rhythm monitoring strategies, ventricular arrhythmias (VAs) are increasingly detected in Fontan patients. However, the prognostic implications of VA are poorly understood. We assessed the incidence of VA in Fontan patients and the implications on transplant-free survival.Medical records of Fontan patients seen at a single center between 2002 and 2019 were reviewed to identify post-Fontan VA (nonsustained ventricular tachycardia >4 beats or sustained >30 seconds). Patients with preFontan VA were excluded. Hemodynamically unstable VA was defined as malignant VA. The primary outcome was death or heart transplantation. Death with censoring at transplant was a secondary outcome.Of 431 Fontan patients, transplant-free survival was 82% at 15 years post-Fontan with 64 (15%) meeting primary outcome of either death (n=16, 3.7%), at a median 4.6 (0.4-10.2) years post-Fontan, or transplant (n=48, 11%), at a median of 11.1 (5.9-16.2) years post-Fontan. Forty-eight (11%) patients were diagnosed with VA (90% nonsustained ventricular tachycardia, 10% sustained ventricular tachycardia). Malignant VA (n=9, 2.0%) was associated with younger age, worse systolic function, and valvular regurgitation. Risk for VA increased with time from Fontan, 2.4% at 10 years to 19% at 20 years. History of Stage 1 surgery with right ventricular to pulmonary artery conduit and older age at Fontan were significant risk factors for VA. VA was strongly associated with an increased risk of transplant or death (HR, 9.2 [95% CI, 4.5-18.7]; P<0.001), with a transplant-free survival of 48% at 5-year post-VA diagnosis.Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis. Risk factors for VA included older age at Fontan and history of right ventricular to pulmonary artery conduit. A diagnosis of VA in Fontan patients should prompt increased clinical surveillance.

  View details for DOI 10.1161/CIRCEP.122.011143

  View details for PubMedID 37254747

• One-Year Outcomes in a Pooled Cohort of Harmony Transcatheter Pulmonary Valve Clinical Trial Participants. JACC. Cardiovascular interventions Gillespie, M. J., McElhinney, D. B., Jones, T. K., Levi, D. S., Asnes, J., Gray, R. G., Cabalka, A. K., Fujimoto, K., Qureshi, A. M., Justino, H., Bergersen, L., Benson, L. N., Haugan, D., Boe, B. A., Cheatham, J. P. 2023

  Abstract

  BACKGROUND: The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration-approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT).OBJECTIVES: One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients.METHODS: Eligible patients had severe PR by echocardiography or PR fraction≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n=42) or TPV25 (n=45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately.RESULTS: In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 groupand 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient > 40mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately.CONCLUSIONS: The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability.

  View details for DOI 10.1016/j.jcin.2023.03.002

  View details for PubMedID 37278682

• Improved Right Ventricular Energy Efficiency by 4-Dimensional Flow Magnetic Resonance Imaging After Harmony Valve Implantation. JACC. Advances Woo, J. P., Dong, M. L., Kong, F., McElhinney, D. B., Schiavone, N., Chan, F., Lui, G. K., Haddad, F., Bernstein, D., Marsden, A. 2023; 2 (3)

  View details for DOI 10.1016/j.jacadv.2023.100284

  View details for PubMedID 37691969

  View details for PubMedCentralID PMC10487049

• Serial Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. World journal for pediatric & congenital heart surgery Wise-Faberowski, L., Long, J., Ma, M., Nadel, H. R., Shek, J., Feinstein, J. A., Martin, E., Hanley, F. L., McElhinney, D. B. 2023: 21501351231162959

  Abstract

  BACKGROUND: In patients with tetralogy of Fallot and major aortopulmonary collaterals (MAPCAs), pulmonary blood supply is highly variable. Our approach to this condition emphasizes complete unifocalization of the pulmonary circulation, incorporating all lung segments and addressing stenoses out to the segmental level. Post-repair, we recommend serial lung perfusion scintigraphy (LPS) to assess short-term changes in pulmonary blood flow distribution.METHODS: We reviewed post-discharge and follow-up LPS performed through three years post-repair and analyzed serial changes in perfusion, risk factors for change, and the relationship between LPS parameters and pulmonary artery reintervention.RESULTS: Of 543 patients who had postoperative LPS results in our system, 317 (58%) had only a predischarge LPS available for review, while 226 had 1 (20%) or more (22%) follow-up scans within three years. Overall, pulmonary flow distribution prior to discharge was balanced, and there was minimal change over time; however, there was considerable patient-to-patient variation in both metrics. On multivariable mixed modeling, time after repair (P=.025), initial anatomy consisting of a ductus arteriosus to one lung (P<.001), and age at repair (P=.014) were associated with changes on serial LPS. Patients who had follow-up LPS were more likely to undergo pulmonary artery reintervention, but within that cohort, LPS parameters were not associated with reintervention risk.CONCLUSION: Serial LPS during the first year after MAPCAs repair is a noninvasive method of screening for significant post-repair pulmonary artery stenosis that occurs in a small but important minority of patients. In patients who received follow-up LPS beyond the perioperative period, there was minimal change over time in the population overall, but large changes in some patients and considerable variability. There was no statistical association between LPS findings and pulmonary artery reintervention.

  View details for DOI 10.1177/21501351231162959

  View details for PubMedID 36972512

• Decoupling Transmission and Transduction for Improved Durability of Highly Stretchable, Soft Strain Sensing: Applications in Human Health Monitoring. Sensors (Basel, Switzerland) Kight, A., Pirozzi, I., Liang, X., McElhinney, D. B., Han, A. K., Dual, S. A., Cutkosky, M. 2023; 23 (4)

  Abstract

  This work presents a modular approach to the development of strain sensors for large deformations. The proposed method separates the extension and signal transduction mechanisms using a soft, elastomeric transmission and a high-sensitivity microelectromechanical system (MEMS) transducer. By separating the transmission and transduction, they can be optimized independently for application-specific mechanical and electrical performance. This work investigates the potential of this approach for human health monitoring as an implantable cardiac strain sensor for measuring global longitudinal strain (GLS). The durability of the sensor was evaluated by conducting cyclic loading tests over one million cycles, and the results showed negligible drift. To account for hysteresis and frequency-dependent effects, a lumped-parameter model was developed to represent the viscoelastic behavior of the sensor. Multiple model orders were considered and compared using validation and test data sets that mimic physiologically relevant dynamics. Results support the choice of a second-order model, which reduces error by 73% compared to a linear calibration. In addition, we evaluated the suitability of this sensor for the proposed application by demonstrating its ability to operate on compliant, curved surfaces. The effects of friction and boundary conditions are also empirically assessed and discussed.

  View details for DOI 10.3390/s23041955

  View details for PubMedID 36850551

  View details for PubMedCentralID PMC9967534

• Improved Right Ventricular Energy Efficiency by 4-Dimensional Flow Magnetic Resonance Imaging After Harmony Valve Implantation JACC:Advances Woo, J. P., Dong, M. L., Kong, F., McElhinney, D. B., Schiavone, N., Chan, F., Lui, G. K., Haddad, F., Bernstein, D., Marsden, A. 2023; 2 (3)

  View details for DOI 10.1016/j.jacadv.2023.100284

• Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable? Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Ma, M., Martin, E., Algaze, C., Collins, R. T., McElhinney, D., Mainwaring, R., Hanley, F. 2023; 26: 2-8

  Abstract

  Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results.

  View details for DOI 10.1053/j.pcsu.2022.12.003

  View details for PubMedID 36842795

• One-Year Outcomes in an Expanded Cohort of Harmony Transcatheter Pulmonary Valve Recipients Asnes, J., Levi, D. S., Gillespie, M. J., Boe, B., McElhinney, D. B., Jones, T. K., Gray, R. G., Cabalka, A. K., Fujimoto, K., Qureshi, A. M., Justino, H., Bergersen, L., Benson, L. N., Haugan, D., Cheatham, J. P. SPRINGER. 2022: 2002

  View details for Web of Science ID 000871235500143

• Ventricular arrhythmias following transcatheter pulmonary valve replacement with the harmony TPV25 device. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Taylor, A., Yang, J., Dubin, A., Chubb, M. H., Motonaga, K., Goodyer, W., Giacone, H., Peng, L., Romfh, A., McElhinney, D., Ceresnak, S. 2022

  Abstract

  BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the Harmony valve (Medtronic, Inc.) was recently approved to treat postoperative native outflow tract pulmonary regurgitation. While the 22mm Harmony valve Early Feasibility Study demonstrated ventricular tachycardia (VT) in only 5% of patients, little is known about ventricular arrhythmias after TPVR with the larger 25mm valve (TPV25).METHODS: A single center review was performed of patients with TPV25 implant from 2020 to 2021. Demographic, cardiac, procedural, and postimplant cardiac telemetry data were collected and compared between patients who did and did not have peri-implant ventricular arrhythmia.RESULTS: Thirty patients underwent TPV25 at a median age of 30 years. On postimplant telemetry, VT events were documented in 12 patients (40%); 11 nonsustained VT (NSVT) (median 3 episodes per patient and 6 beats per episode, maximum 157 episodes) and 1 sustained VT (3%), with Torsades de Pointes secondary to a short coupled premature ventricular contraction (PVC). VT events were associated with annular valve positioning (p<0.001) and increased postimplant PVC burden (p<0.0001), but there was no association between VT and other demongraphic, historical, or procedural factors. The frequency of NSVT events fell from 3/hfrom 0 to 12h postimplant to 0.5/hr from 12 to 24h (p<0.001).CONCLUSION: VT occurred commonly (40%) in the first 24h after TPV25 implant, with self-limited NSVT in 11 of 12 patients and 1 patient with cardiac arrest secondary to Torsades de Pointes. VT only occurred with annular valve positioning. Larger, longer-term studies are needed to determine risk factors for and natural history of post-TPVR VT.

  View details for DOI 10.1002/ccd.30393

  View details for PubMedID 36198126

• Management of Infective Endocarditis in People Who Inject Drugs: A Scientific Statement From the American Heart Association CIRCULATION Baddour, L. M., Weimer, M. B., Wurcel, A. G., McElhinney, D. B., Marks, L. R., Fanucchi, L. C., Garrigos, Z., Pettersson, G. B., DeSimone, D. C., Council Lifelong Congenital Heart, Council Cardiovasc Surg Anesthesia, Council Cardiovasc Stroke Nursing, Council Clinical Cardiology, Council Peripheral Vasc Dis 2022; 146 (14): E187-E201

  Abstract

  The American Heart Association has sponsored both guidelines and scientific statements that address the diagnosis, management, and prevention of infective endocarditis. As a result of the unprecedented and increasing incidence of infective endocarditis cases among people who inject drugs, the American Heart Association sponsored this original scientific statement. It provides a more in-depth focus on the management of infective endocarditis among this unique population than what has been provided in prior American Heart Association infective endocarditis-related documents.A writing group was named and consisted of recognized experts in the fields of infectious diseases, cardiology, addiction medicine, and cardiovascular surgery in October 2021. A literature search was conducted in Embase on November 19, 2021, and multiple terms were used, with 1345 English-language articles identified after removal of duplicates.Management of infective endocarditis in people who inject drugs is complex and requires a unique approach in all aspects of care. Clinicians must appreciate that it requires involvement of a variety of specialists and that consultation by addiction-trained clinicians is as important as that of more traditional members of the endocarditis team to improve infective endocarditis outcomes. Preventive measures are critical in people who inject drugs and are cured of an initial bout of infective endocarditis because they remain at extremely high risk for subsequent bouts of infective endocarditis, regardless of whether injection drug use is continued.

  View details for DOI 10.1161/CIR.0000000000001090

  View details for Web of Science ID 000886562300001

  View details for PubMedID 36043414

• Single center blind testing of a US multi-center validated diagnostic algorithm for Kawasaki disease in Taiwan. Frontiers in immunology Kuo, H. C., Hao, S., Jin, B., Chou, C. J., Han, Z., Chang, L. S., Huang, Y. H., Hwa, K., Whitin, J. C., Sylvester, K. G., Reddy, C. D., Chubb, H., Ceresnak, S. R., Kanegaye, J. T., Tremoulet, A. H., Burns, J. C., McElhinney, D., Cohen, H. J., Ling, X. B. 2022; 13: 1031387

  Abstract

  Kawasaki disease (KD) is the leading cause of acquired heart disease in children. The major challenge in KD diagnosis is that it shares clinical signs with other childhood febrile control (FC) subjects. We sought to determine if our algorithmic approach applied to a Taiwan cohort.A single center (Chang Gung Memorial Hospital in Taiwan) cohort of patients suspected with acute KD were prospectively enrolled by local KD specialists for KD analysis. Our previously single-center developed computer-based two-step algorithm was further tested by a five-center validation in US. This first blinded multi-center trial validated our approach, with sufficient sensitivity and positive predictive value, to identify most patients with KD diagnosed at centers across the US. This study involved 418 KDs and 259 FCs from the Chang Gung Memorial Hospital in Taiwan.Our diagnostic algorithm retained sensitivity (379 of 418; 90.7%), specificity (223 of 259; 86.1%), PPV (379 of 409; 92.7%), and NPV (223 of 247; 90.3%) comparable to previous US 2016 single center and US 2020 fiver center results. Only 4.7% (15 of 418) of KD and 2.3% (6 of 259) of FC patients were identified as indeterminate. The algorithm identified 18 of 50 (36%) KD patients who presented 2 or 3 principal criteria. Of 418 KD patients, 157 were infants younger than one year and 89.2% (140 of 157) were classified correctly. Of the 44 patients with KD who had coronary artery abnormalities, our diagnostic algorithm correctly identified 43 (97.7%) including all patients with dilated coronary artery but one who found to resolve in 8 weeks.This work demonstrates the applicability of our algorithmic approach and diagnostic portability in Taiwan.

  View details for DOI 10.3389/fimmu.2022.1031387

  View details for PubMedID 36263040

  View details for PubMedCentralID PMC9575935

• Machine Learning for Pediatric Echocardiographic Mitral Regurgitation Detection. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Edwards, L. A., Feng, F., Iqbal, M., Fu, Y., Sanyahumbi, A., Hao, S., McElhinnney, D. B., Ling, X. B., Sable, C., Luo, J. 2022

  Abstract

  Echocardiography-based screening for valvular disease in at-risk asymptomatic children can result in early diagnosis. These screening programs, however, are resource intensive, and may not be feasible in many resource-limited settings. Automated echocardiographic diagnosis may enable more widespread echocardiographic screening, early diagnosis, and improved outcomes. In this feasibility study, we sought to build a machine learning model capable of identifying mitral regurgitation (MR) on echocardiogram.Echocardiograms were labeled by clip for view and by frame for the presence of MR. The labeled data were used to build two convolutional neural networks (CNNs) to perform the stepwise tasks of classifying the clips 1) by view and 2) by the presence of any MR, including physiologic, in parasternal long axis color Doppler views (PLAX-C). We developed the view classification model using 66,330 frames and evaluated model performance using a hold-out testing dataset with 45 echocardiograms (11,730 frames). We developed the MR detection model using 938 frames and evaluated model performance using a hold-out testing dataset with 42 echocardiograms (182 frames). Metrics to evaluate model performance included accuracy, precision, recall, F1 score (average of precision and recall, 0 to 1 with 1 suggesting perfect precision and recall), and receiver-operating characteristic analysis.For the PLAX-C view, the view classification CNN achieved an F1 score of 0.97. The MR detection CNN achieved a testing accuracy of 0.86 and an area under the receiver operating characteristic curve of 0.91.A machine learning model is capable of discerning MR on transthoracic echocardiography. This is an encouraging step toward machine learning-based diagnosis of valvular heart disease on pediatric echocardiograms.

  View details for DOI 10.1016/j.echo.2022.09.017

  View details for PubMedID 36191670

• Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation. Journal of the American College of Cardiology Chubb, H., Bulic, A., Mah, D., Moore, J. P., Janousek, J., Fumanelli, J., Asaki, S. Y., Pflaumer, A., Hill, A. C., Escudero, C., Kwok, S. Y., Mangat, J., Ochoa Nunez, L. A., Balaji, S., Rosenthal, E., Regan, W., Horndasch, M., Asakai, H., Tanel, R., Czosek, R. J., Young, M., Bradley, D. J., Paul, T., Fischbach, P., Malloy-Walton, L., McElhinney, D. B., Dubin, A. M. 2022; 80 (9): 902-914

  Abstract

  BACKGROUND: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPMv) may be associated with additional adverse long-term outcomes.OBJECTIVES: The goal of this study was to quantify the attributable risk of PPMv in patients with SV, and to identify modifiable risk factors.METHODS: This international study was sponsored by the Pediatric and Congenital Electrophysiology Society. Centers contributed baseline and longitudinal data for functionally SV patients with PPMv. Enrollment was at implantation. Controls were matched 1:1 to PPMv subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death.RESULTS: In total, 236 PPMv subjects and 213 matched controls were identified (22 centers, 9 countries). Median age at enrollment was 5.3 years (quartiles: 1.5-13.2 years), follow-up 6.9 years (3.4-11.6 years). Median percent ventricular pacing (Vp) was 90.8% (25th-75th percentile: 4.3%-100%) in the PPMv cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPMv was 3.8 (95%CI 1.9-7.6; P< 0.001). Within the PPMv population, higher Vp (HR: 1.009 per %; P=0.009), higher QRS z-score (HR: 1.19; P=0.009) and nonapical lead position (HR: 2.17; P=0.042) were all associated with death/transplantation.CONCLUSIONS: PPMv in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPMv cohort. Increased Vp, higher QRS z-score, and nonapical ventricular lead position are all associated with higher risk of adverse outcome and may be modifiable risk factors.

  View details for DOI 10.1016/j.jacc.2022.05.053

  View details for PubMedID 36007989

• Innominate artery patency after direct cannulation in neonates. JTCVS techniques Choi, P. S., Nasirov, T., Hanley, F., Peng, L., McElhinney, D. B., Ma, M. 2022; 14: 171-176

  Abstract

  Objective: The study objective was to determine the short-term incidence of innominate artery stenosis for neonates who underwent direct innominate artery cannulation during the Norwood procedure.Methods: This is a retrospective, single-institution review of 92 patients who underwent the Norwood procedure with direct innominate artery cannulation from 2006 to 2017. The primary outcome was angiographic evidence of patency at pre-Glenn cardiac catheterization. Patient characteristics, intraoperative surgical and hemodynamic measurements, and postoperative neurologic findings were recorded.Results: At a median age of 5.0days, 92 neonates underwent the Norwood procedure with direct innominate artery cannulation. These patients underwent cardiac catheterization at a median of 3.0months after the index operation. In 5 of 92 patients with catheterization images available for review, there was angiographic evidence of mild innominate artery stenosis, and none had moderate or severe stenosis. Review of follow-up records did not reveal evidence of clinically significant stenosis or innominate artery reintervention.Conclusions: In neonates undergoing the Norwood procedure with direct innominate cannulation, innominate artery stenosis was uncommon and clinically significant stenosis did not occur.

  View details for DOI 10.1016/j.xjtc.2022.06.001

  View details for PubMedID 35967223

• Elucidating tricuspid Doppler signal interpolation and its implication for assessing pulmonary hypertension PULMONARY CIRCULATION Dual, S. A., Verdonk, C., Amsallem, M., Pham, J., Obasohan, C., Nataf, P., McElhinney, D. B., Arunamata, A., Kuznetsova, T., Zamanian, R., Feinstein, J. A., Marsden, A., Haddad, F. 2022; 12 (3): e12125

  Abstract

  Doppler echocardiography plays a central role in the assessment of pulmonary hypertension (PAH). We aim to improve quality assessment of systolic pulmonary arterial pressure (SPAP) by applying a cubic polynomial interpolation to digitized tricuspid regurgitation (TR) waveforms. Patients with PAH and advanced lung disease were divided into three cohorts: a derivation cohort (n = 44), a validation cohort (n = 71), an outlier cohort (n = 26), and a non-PAH cohort (n = 44). We digitized TR waveforms and analyzed normalized duration, skewness, kurtosis, and first and second derivatives of pressure. Cubic polynomial interpolation was applied to three physiology-driven phases: the isovolumic phase, ejection phase, and "shoulder" point phase. Coefficients of determination and a Bland-Altman analysis was used to assess bias between methods. The cubic polynomial interpolation of the TR waveform correlated strongly with expert read right ventricular systolic pressure (RVSP) with R 2 > 0.910 in the validation cohort. The biases when compared to invasive SPAP measured within 24 h were 6.03 [4.33; 7.73], -2.94 [1.47; 4.41], and -3.11 [-4.52; -1.71] mmHg, for isovolumic, ejection, and shoulder point interpolations, respectively. In the outlier cohort with more than 30% difference between echocardiographic estimates and invasive SPAP, cubic polynomial interpolation significantly reduced underestimation of RVSP. Cubic polynomial interpolation of the TR waveform based on isovolumic or early ejection phase may improve RVSP estimates.

  View details for DOI 10.1002/pul2.12125

  View details for Web of Science ID 000843054900001

  View details for PubMedID 36016669

  View details for PubMedCentralID PMC9395694

• REPLY: Risk of Endocarditis Extension in Ross Reintervention With Transcatheter Pulmonary Valve Replacement JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Ewert, P., Schubert, S. 2022; 79 (21): E459

  View details for DOI 10.1016/j.jacc.2022.03.360

  View details for Web of Science ID 000830272100002

  View details for PubMedID 35618355

• Distance from home to birth hospital, transfer, and mortality in neonates with hypoplastic left heart syndrome in California. Birth defects research Purkey, N. J., Ma, C., Lee, H. C., Hintz, S. R., Shaw, G. M., McElhinney, D. B., Carmichael, S. L. 2022

  Abstract

  BACKGROUND: Prior studies report a lower risk of mortality among neonates with hypoplastic left heart syndrome (HLHS) who are born at a cardiac surgical center, but many neonates with HLHS are born elsewhere and transferred for repair. We investigated the associations between the distance from maternal home to birth hospital, the need for transfer after birth, sociodemographic factors, and mortality in infants with HLHS in California from 2006 to 2011.METHODS: We used linked data from two statewide databases to identify neonates for this study. Three groups were included in the analysis: "lived close/not transferred," "lived close/transferred," and "lived far/not transferred." We defined "lived close" versus "lived far" as 11miles, the median distance from maternal residence to birth hospital. Log-binomial regression models were used to identify the association between sociodemographic variables, distance to birth hospital and transfer. Cox proportional hazards models were used to identify the association between mortality and distance to birth hospital and transfer. Models were adjusted for sociodemographic variables.RESULTS: Infants in the lived close/not transferred and the lived close/transferred groups (vs. the lived far/not transferred group) were more likely to live in census tracts above the 75th percentile for poverty with relative risks 1.94 (95% confidence interval [CI] 1.41-2.68) and 1.21 (95% CI 1.05-1.40), respectively. Neonatal mortality was higher among the lived close/not transferred group compared with the lived far/not transferred group (hazard ratio 1.77, 95% CI 1.17-2.67).CONCLUSIONS: Infants born to mothers experiencing poverty were more likely to be born close to home. Infants with HLHS who were born close to home and not transferred to a cardiac center had a higher risk of neonatal mortality than infants who were delivered far from home and not transferred. Future studies should identify the barriers to delivery at a cardiac center for mothers experiencing poverty.

  View details for DOI 10.1002/bdr2.2020

  View details for PubMedID 35488460

• Gestational Dating by Urine Metabolic Profile at High Resolution Weekly Sampling Timepoints: Discovery and Validation. Frontiers in molecular medicine Sylvester, K. G., Hao, S., Li, Z., Han, Z., Tian, L., Ladella, S., Wong, R. J., Shaw, G. M., Stevenson, D. K., Cohen, H. J., Whitin, J. C., McElhinney, D. B., Ling, X. B. 2022; 2: 844280

  Abstract

  Background: Pregnancy triggers longitudinal metabolic alterations in women to allow precisely-programmed fetal growth. Comprehensive characterization of such a "metabolic clock" of pregnancy may provide a molecular reference in relation to studies of adverse pregnancy outcomes. However, a high-resolution temporal profile of metabolites along a healthy pregnancy remains to be defined. Methods: Two independent, normal pregnancy cohorts with high-density weekly urine sampling (discovery: 478 samples from 19 subjects at California; validation: 171 samples from 10 subjects at Alabama) were studied. Urine samples were profiled by liquid chromatography-mass spectrometry (LC-MS) for untargeted metabolomics, which was applied for gestational age dating and prediction of time to delivery. Results: 5,473 urinary metabolic features were identified. Partial least-squares discriminant analysis on features with robust signals (n = 1,716) revealed that the samples were distributed on the basis of the first two principal components according to their gestational age. Pathways of bile secretion, steroid hormone biosynthesis, pantohenate, and CoA biosynthesis, benzoate degradation, and phenylpropanoid biosynthesis were significantly regulated, which was collectively applied to discover and validate a predictive model that accurately captures the chronology of pregnancy. With six urine metabolites (acetylcholine, estriol-3-glucuronide, dehydroepiandrosterone sulfate, α-lactose, hydroxyexanoy-carnitine, and l-carnitine), models were constructed based on gradient-boosting decision trees to date gestational age in high accordance with ultrasound results, and to accurately predict time to delivery. Conclusion: Our study characterizes the weekly baseline profile of the human pregnancy metabolome, which provides a high-resolution molecular reference for future studies of adverse pregnancy outcomes.

  View details for DOI 10.3389/fmmed.2022.844280

  View details for PubMedID 39086969

  View details for PubMedCentralID PMC11285704

• VENTRICULAR ARRHYTHMIAS FOLLOWING TRANSCATHETER PULMONARY VALVE REPLACEMENT WITH THE HARMONY(C) TPV 25 DEVICE Taylor, A., Yang, J., Dubin, A., Chubb, M., Motonaga, K., Goodyer, W., Giacone, H., Peng, L. F., Romfh, A. W., McElhinney, D. B., Ceresnak, S. R. ELSEVIER SCIENCE INC. 2022: 1362

  View details for Web of Science ID 000781026601461

• Airway Characteristics of Patients With 22q11 Deletion Undergoing Pulmonary Artery Reconstruction Surgery: Retrospective Cohort Study. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Sganga, D., Meister, K., Sidell, D. R., Wise-Faberowski, L., Shek, J., Ma, M., Martin, E., Hanley, F. L., McElhinney, D., Asija, R. 2022

  Abstract

  OBJECTIVES: We have previously shown that patients with a chromosome 22q11 microdeletion are at risk for prolonged respiratory failure after pulmonary artery reconstruction surgery compared with those with normal genotype. We sought to describe preexisting airway abnormalities in this patient population and examine relationships between airway abnormalities and outcomes.DESIGN: Single-center retrospective chart review from Society of Thoracic Surgery and Pediatric Cardiac Critical Care Consortium databases and the electronic medical record.SETTING: Lucile Packard Children's Hospital at Stanford from September 2017 to February 2019.PATIENTS: All patients undergoing pulmonary artery reconstruction surgery were considered for inclusion.INTERVENTIONS: We identified 127 patients meeting study inclusion criteria. Thirty-nine patients met specific criteria and underwent screening preoperative bronchoscopy including microdirect laryngoscopy and lower airway examination. Postoperative bronchoscopy was performed at the discretion of the intensive care team.MEASUREMENTS AND MAIN RESULTS: Airway abnormalities were detected in 25/26 of children (96%) with a chromosome 22q11 deletion who underwent preoperative bronchoscopy. Upper and lower airway pathologies were found in 19/25 (73%) and 21/25 (81%) patients, respectively, and it was common for patients to have more than one abnormality. Presence of 22q11 deletion was associated with longer duration of mechanical ventilation (9.1 vs 4.3 d; p = 0.001), use of noninvasive positive pressure support (13 vs 6 d; p = 0.001), and longer hospital stays (30 vs 14 d; p = 0.002). These outcomes were worse when compared with patients with known airway abnormalities who did not have 22q11 deletion.CONCLUSIONS: Preexisting upper and lower airway pathologies are common in patients with a chromosome 22q11 deletion who undergo pulmonary artery reconstruction surgery. Despite similar postoperative hemodynamics and outcomes as their counterparts without 22q11 deletion, 22q11 deletion is associated with more postoperative respiratory complications not entirely explained by preexisting airway abnormalities.

  View details for DOI 10.1097/PCC.0000000000002921

  View details for PubMedID 35213412

• Classic pattern dyssynchrony is associated with outcome in patients with Fontan circulation. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Rosner, A., McElhinney, D. B., Diab, S. G., Friedberg, M. K., Lui, G. K. 2022

  Abstract

  BACKGROUND: Morbidity and mortality increase as Fontan patients age into adulthood. Limited studies have examined cardiac magnetic resonance and echocardiographic parameters to predict death and transplantation in children after Fontan operation. The aim of the study was to investigate echocardiographic parameters in adolescents and adults after Fontan operation including myocardial mechanics including classic pattern dyssynchrony (CPD) as predictors of transplantation or death.METHODS: In a cross-sectional retrospective study, strain analysis was performed on echocardiography studies performed between 2001 and 2015 of 110 patients with single ventricle physiology after the Fontan procedure. Strain curves were measured and visually assessed for the presence of CPD. The primary end point was death or transplantation after a follow-up period of 85±35 months after echocardiography.RESULTS: Median age at date of echocardiography was 20, range 3 to 45 years. Of 110 patients 28 patients were transplanted. During the study-period 3 patients died after transplantation and 7 patients died without being transplanted. CPD was seen in 16 and protein losing enteropathy (PLE) in 21 of 110 patients. By multivariate-analysis, CPD (HR 9.4 CI 2.6-34.6), PLE (HR 10.6 CI 3.4-33.2); systolic blood pressure (HR 0.954 CI 0.913-0.996), systolic/diastolic duration ratio (HR 6.83 CI 1.33-35.0) and E wave deceleration time (HR 0.98 CI 0.97 - 0.99) were independently associated with the primary end point.CONCLUSION: CPD, PLE, systolic and diastolic ventricular dysfunction are significantly associated with transplantation or death in Fontan operated patients. In selected patients, the presence of CPD may be a basis to investigate cardiac resynchronization therapy as a treatment strategy.

  View details for DOI 10.1016/j.echo.2022.01.012

  View details for PubMedID 35121052

• Relation Between Pulmonary Artery Pressures Measured Intraoperatively and at One-Year Catheterization After Unifocalization and Repair of Tetralogy with Major Aortopulmonary Collateral Arteries. Seminars in thoracic and cardiovascular surgery Ma, M., Peng, L. F., Zhang, Y., Wise-Faberowski, L., Martin, E., Hanley, F. L., McElhinney, D. B. 1800

  Abstract

  To assess the relationships between pulmonary artery (PA) pressure and the PA:aortic systolic pressure ratio measured intraoperatively and at surveillance catheterization in patients achieving complete unifocalization and repair for tetralogy of Fallot with major aortopulmonary collateral arteries (TOF/MAPCAs). This was a single-center retrospective cohort analysis of all patients who underwent complete repair of TOF/MAPCAs from 2002-2019 and received a postoperative surveillance catheterization at our center 6-24 months after surgery. Associations between intraoperative and catheter hemodynamic data were analyzed. 163 patients were included. Median systolic PA pressure was 30 (quartiles 26, 35) and 35 (28, 42) mmHg intraoperatively and at catherization respectively; systolic aortic pressure 90 (86, 100) and 84 (76, 92); and PA:aortic pressure ratio was 0.33 (0.28, 0.40) and 0.41 (0.34, 0.49). Moderate correlation was found between the intraoperative and catheter-based hemodynamics, with the majority of systolic PA pressures within 10mmHg and PA:Ao systolic ratios within 0.1. Changes in the ratio were influenced to a similar degree by differences in PA and aortic pressures. Surgical and/or catheter reinterventions were more common in patients with both higher intraoperative PA systolic pressure and PA:aortic systolic ratios and in those with greater discrepancy between intraoperative and catheterization values. PA systolic pressure and the PA:aortic systolic pressure ratio measured immediately after repair remain useful metrics for assessing the initial operative PA reconstruction, and as indicators of longer term hemodynamics. Initially elevated and subsequently discrepant PA systolic pressure and PA:aortic systolic pressure ratios were associated with higher rates of reintervention. (Figure 7).

  View details for DOI 10.1053/j.semtcvs.2022.01.004

  View details for PubMedID 35092847

• Reintervention and Survival After Transcatheter Pulmonary Valve Replacement. Journal of the American College of Cardiology McElhinney, D. B., Zhang, Y., Levi, D. S., Georgiev, S., Biernacka, E. K., Goldstein, B. H., Shahanavaz, S., Qureshi, A. M., Cabalka, A. K., Bauser-Heaton, H., Torres, A. J., Morray, B. H., Armstrong, A. K., Millan-Iturbe, O., Peng, L. F., Aboulhosn, J. A., Rużyłło, W., Berger, F., Sondergaard, L., Schranz, D., Cheatham, J. P., Jones, T. K., Ewert, P., Schubert, S. 2022; 79 (1): 18-32

  Abstract

  Transcatheter pulmonary valve (TPV) replacement (TPVR) has become the standard therapy for postoperative pulmonary outflow tract dysfunction in patients with a prosthetic conduit/valve, but there is limited information about risk factors for death or reintervention after this procedure.This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort.International registry focused on time-related outcomes after TPVR.Investigators submitted data for 2,476 patients who underwent TPVR and were followed up for 8,475 patient-years. A total of 95 patients died after TPVR, most commonly from heart failure (n = 24). The cumulative incidence of death was 8.9% (95% CI: 6.9%-11.5%) 8 years after TPVR. On multivariable analysis, age at TPVR (HR: 1.04 per year; 95% CI: 1.03-1.06 per year; P < 0.001), a prosthetic valve in other positions (HR: 2.1; 95% CI: 1.2-3.7; P = 0.014), and an existing transvenous pacemaker/implantable cardioverter-defibrillator (HR: 2.1; 95% CI: 1.3-3.4; P = 0.004) were associated with death. A total of 258 patients underwent TPV reintervention. At 8 years, the cumulative incidence of any TPV reintervention was 25.1% (95% CI: 21.8%-28.5%) and of surgical TPV reintervention was 14.4% (95% CI: 11.9%-17.2%). Risk factors for surgical reintervention included age (0.95 per year [95% CI: 0.93-0.97 per year]; P < 0.001), prior endocarditis (2.5 [95% CI: 1.4-4.3]; P = 0.001), TPVR into a stented bioprosthetic valve (1.7 [95% CI: 1.2-2.5]; P = 0.007), and postimplant gradient (1.4 per 10 mm Hg [95% CI: 1.2-1.7 per 10 mm Hg]: P < 0.001).These findings support the conclusion that survival and freedom from reintervention or surgery after TPVR are generally comparable to outcomes of surgical conduit/valve replacement across a wide age range.

  View details for DOI 10.1016/j.jacc.2021.10.031

  View details for PubMedID 34991785

• Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial. Circulation. Cardiovascular interventions Jones, T. K., McElhinney, D. B., Vincent, J. A., Hellenbrand, W. E., Cheatham, J. P., Berman, D. P., Zahn, E. M., Khan, D. M., Rhodes, J. F., Weng, S., Bergersen, L. J. 1800: CIRCINTERVENTIONS121010852

  Abstract

  BACKGROUND: The Melody valve was developed to extend the useful life of previously implanted right ventricular outflow tract (RVOT) conduits or bioprosthetic pulmonary valves, while preserving RV function and reducing the lifetime burden of surgery for patients with complex congenital heart disease.METHODS: Enrollment for the US Investigational Device Exemption study of the Melody valve began in 2007. Extended follow-up was completed in 2020. The primary outcome was freedom from transcatheter pulmonary valve (TPV) dysfunction (freedom from reoperation, reintervention, moderate or severe pulmonary regurgitation, and/or mean RVOT gradient >40 mmHg). Secondary end points included stent fracture, catheter reintervention, surgical conduit replacement, and death.RESULTS: One hundred seventy-one subjects with RVOT conduit or bioprosthetic pulmonary valve dysfunction were enrolled. One hundred fifty underwent Melody TPV replacement. Median age was 19 years (Q1-Q3: 15-26). Median discharge mean RVOT Doppler gradient was 17 mm Hg (Q1-Q3: 12-22). The 149 patients implanted >24 hours were followed for a median of 8.4 years (Q1-Q3: 5.4-10.1). At 10 years, estimated freedom from mortality was 90%, from reoperation 79%, and from any reintervention 60%. Ten-year freedom from TPV dysfunction was 53% and was significantly shorter in children than in adults. Estimated freedom from TPV-related endocarditis was 81% at 10 years (95% CI, 69%-89%), with an annualized rate of 2.0% per patient-year.CONCLUSIONS: Ten-year outcomes from the Melody Investigational Device Exemption trial affirm the benefits of Melody TPV replacement in the lifetime management of patients with RVOT conduits and bioprosthetic pulmonary valves by providing sustained symptomatic and hemodynamic improvement in the majority of patients.REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT00740870.

  View details for DOI 10.1161/CIRCINTERVENTIONS.121.010852

  View details for PubMedID 34930015

• Mid-Term Outcomes from the Harmony Transcatheter Pulmonary Valve Pivotal Trial and Continued Access Study Gillespie, M., McElhinney, D., Jones, T., Levi, D., Weng, S., Cheatham, J. SPRINGER. 2021: 1903

  View details for Web of Science ID 000712979800056

• Early-pregnancy prediction of risk for pre-eclampsia using maternal blood leptin/ceramide ratio: discovery and confirmation. BMJ open Huang, Q., Hao, S., You, J., Yao, X., Li, Z., Schilling, J., Thyparambil, S., Liao, W., Zhou, X., Mo, L., Ladella, S., Davies-Balch, S. R., Zhao, H., Fan, D., Whitin, J. C., Cohen, H. J., McElhinney, D. B., Wong, R. J., Shaw, G. M., Stevenson, D. K., Sylvester, K. G., Ling, X. B. 2021; 11 (11): e050963

  Abstract

  OBJECTIVE: This study aimed to develop a blood test for the prediction of pre-eclampsia (PE) early in gestation. We hypothesised that the longitudinal measurements of circulating adipokines and sphingolipids in maternal serum over the course of pregnancy could identify novel prognostic biomarkers that are predictive of impending event of PE early in gestation.STUDY DESIGN: Retrospective discovery and longitudinal confirmation.SETTING: Maternity units from two US hospitals.PARTICIPANTS: Six previously published studies of placental tissue (78 PE and 95 non-PE) were compiled for genomic discovery, maternal sera from 15 women (7 non-PE and 8 PE) enrolled at ProMedDx were used for sphingolipidomic discovery, and maternal sera from 40 women (20 non-PE and 20 PE) enrolled at Stanford University were used for longitudinal observation.OUTCOME MEASURES: Biomarker candidates from discovery were longitudinally confirmed and compared in parallel to the ratio of placental growth factor (PlGF) and soluble fms-like tyrosine kinase (sFlt-1) using the same cohort. The datasets were generated by enzyme-linked immunosorbent and liquid chromatography-tandem mass spectrometric assays.RESULTS: Our discovery integrating genomic and sphingolipidomic analysis identified leptin (Lep) and ceramide (Cer) (d18:1/25:0) as novel biomarkers for early gestational assessment of PE. Our longitudinal observation revealed a marked elevation of Lep/Cer (d18:1/25:0) ratio in maternal serum at a median of 23 weeks' gestation among women with impending PE as compared with women with uncomplicated pregnancy. The Lep/Cer (d18:1/25:0) ratio significantly outperformed the established sFlt-1/PlGF ratio in predicting impending event of PE with superior sensitivity (85% vs 20%) and area under curve (0.92 vs 0.52) from 5 to 25 weeks of gestation.CONCLUSIONS: Our study demonstrated the longitudinal measurement of maternal Lep/Cer (d18:1/25:0) ratio allows the non-invasive assessment of PE to identify pregnancy at high risk in early gestation, outperforming the established sFlt-1/PlGF ratio test.

  View details for DOI 10.1136/bmjopen-2021-050963

  View details for PubMedID 34824115

• Multicenter Study of Endocarditis AfterTranscatheter Pulmonary ValveReplacement. Journal of the American College of Cardiology McElhinney, D. B., Zhang, Y., Aboulhosn, J. A., Morray, B. H., Biernacka, E. K., Qureshi, A. M., Torres, A. J., Shahanavaz, S., Goldstein, B. H., Cabalka, A. K., Bauser-Heaton, H., Georgiev, S., Berger, F., Millan-Iturbe, O., Peng, L. F., Armstrong, A. K., Levi, D. S., Fronczak-Jakubczyk, A., Sondergaard, L., Anderson, J. H., Schranz, D., Jones, T. K., Cheatham, J. P., Schubert, S., Ewert, P. 2021; 78 (6): 575-589

  Abstract

  BACKGROUND: Endocarditis has emerged as one of the most impactful adverse events after transcatheter pulmonary valve replacement (TPVR), but there is limited information about risk factors for and outcomes of this complication.OBJECTIVES: The purpose of this study was to evaluate risk factors for and outcomes of endocarditis in a large multicenter cohort.METHODS: The authors established an international registry focused on characterizing endocarditis after TPVR, including the incidence, risk factors, characteristics, and outcomes.RESULTS: Investigators submitted data for 2,476 patients who underwent TPVR between July 2005 and March 2020 and were followed for 8,475 patient-years. In total, 182 patients were diagnosed with endocarditis a median of 2.7 years after TPVR, for a cumulative incidence of 9.5% (95% CI: 7.9%-11.1%) at 5 years and 16.9% (95% CI: 14.2%-19.8%) at 8 years (accounting for competing risks: death, heart transplant, and explant) and an annualized incidence of 2.2 per 100 patient-years. Staphylococcus aureus and Viridans group Streptococcus species together accounted for 56% of cases. Multivariable analysis confirmed that younger age, a previous history of endocarditis, and a higher residual gradient were risk factors for endocarditis, but transcatheter pulmonary valve type was not. Overall, right ventricular outflow tract (RVOT) reintervention was less often to treat endocarditis than for other reasons, but valve explant was more often caused by endocarditis. Endocarditis was severe in 44% of patients, and 12 patients (6.6%) died, nearly all of whom were infected with Staphylococcus aureus.CONCLUSIONS: The incidence of endocarditis in this multicenter registry was constant over time and consistent with prior smaller studies. The findings of this study, along with ongoing efforts to understand and mitigate risk, will be critical to improve the lifetime management of patients with heart disease involving the RVOT. Although endocarditis can be a serious adverse outcome, TPVR remains an important tool in the management of RVOT dysfunction.

  View details for DOI 10.1016/j.jacc.2021.05.044

  View details for PubMedID 34353535

• Clinical and hemodynamic characteristics of the pediatric failing Fontan. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Dykes, J. C., Rosenthal, D. N., Bernstein, D., McElhinney, D. B., Chrisant, M. R., Daly, K. P., Ameduri, R. K., Knecht, K., Richmond, M. E., Lin, K. Y., Urschel, S., Simmonds, J., Simpson, K. E., Albers, E. L., Khan, A., Schumacher, K., Almond, C. S., Chen, S., Pediatric Heart Transplant Society 2021

  Abstract

  AIM: To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.METHODS: In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013.Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with "normal" hearts (FNH). Primary outcome was waitlist and post-transplant mortality.RESULTS: 177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m2. FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).CONCLUSIONS: Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.

  View details for DOI 10.1016/j.healun.2021.07.017

  View details for PubMedID 34412962

• The challenges of an aging Tetralogy of Fallot population. Expert review of cardiovascular therapy Woo, J. P., McElhinney, D. B., Lui, G. K. 2021

  Abstract

  INTRODUCTION: Advancements in surgery and management have resulted in a growing population of aging adults with tetralogy of Fallot (TOF). As a result, there has been a parallel growth in late complications associated with the sequelae from the underlying cardiac anomalies as well as the surgical and other interventional treatments.AREAS COVERED: Here, we review challenges related to an aging population of patients with TOF, particularly late complications, and highlight advances in management and key areas for future research. Pulmonary regurgitation, heart failure, arrhythmias, and aortic complications are some of these late complications. There is also a growing incidence of acquired cardiovascular disease, obesity, and diabetes associated with aging. Management of these late complications and acquired comorbidities continues to evolve as research provides insights into long-term outcomes from medical therapies and surgical interventions.EXPERT OPINION: The management of an aging TOF population will continue to transform with advances in imaging technologies to identify subclinical disease and valve replacement technologies that will prevent and mitigate disease progression. In coming years, we speculate there will be more data to support the use of novel heart failure therapies in TOF and consensus guidelines on management of refractory arrhythmias and aortic complications.

  View details for DOI 10.1080/14779072.2021.1940960

  View details for PubMedID 34102942

• Updates on transcatheter pulmonary valve replacement PROGRESS IN PEDIATRIC CARDIOLOGY Morray, B. H., McElhinney, D. B. 2021; 61

  View details for DOI 10.1016/j.ppedcard.2021.101345

  View details for Web of Science ID 000652675900012

• Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy. Journal of magnetic resonance imaging : JMRI Dual, S. A., Maforo, N. G., McElhinney, D. B., Prosper, A., Wu, H. H., Maskatia, S., Renella, P., Halnon, N., Ennis, D. B. 2021

  Abstract

  BACKGROUND: Clinical management of boys with Duchenne muscular dystrophy (DMD) relies on in-depth understanding of cardiac involvement, but right ventricular (RV) structural and functional remodeling remains understudied.PURPOSE: To evaluate several analysis methods and identify the most reliable one to measure RV pre- and postcontrast T1 (RV-T1) and to characterize myocardial remodeling in the RV of boys with DMD.STUDY TYPE: Prospective.POPULATION: Boys with DMD (N=27) and age-/sex-matched healthy controls (N=17) from two sites.FIELD STRENGTH/SEQUENCE: 3.0T using balanced steady state free precession, motion-corrected phase sensitive inversion recovery and modified Look-Locker inversion recovery sequences.ASSESSMENT: Biventricular mass (Mi), end-diastolic volume (EDVi) and ejection fraction (EF) assessment, tricuspid annular excursion (TAE), late gadolinium enhancement (LGE), pre- and postcontrast myocardial T1 maps. The RV-T1 reliability was assessed by three observers in four different RV regions of interest (ROI) using intraclass correlation (ICC).STATISTICAL TESTS: The Wilcoxon rank sum test was used to compare RV-T1 differences between DMD boys with negative LGE(-) or positive LGE(+) and healthy controls. Additionally, correlation of precontrast RV-T1 with functional measures was performed. A P-value <0.05 was considered statistically significant.RESULTS: A 1-pixel thick RV circumferential ROI proved most reliable (ICC>0.91) for assessing RV-T1. Precontrast RV-T1 was significantly higher in boys with DMD compared to controls. Both LGE(-) and LGE(+) boys had significantly elevated precontrast RV-T1 compared to controls (1543 [1489-1597] msec and 1550 [1402-1699] msec vs. 1436 [1399-1473] msec, respectively). Compared to healthy controls, boys with DMD had preserved RVEF (51.8 [9.9]% vs. 54.2 [7.2]%, P=0.31) and significantly reduced RVMi (29.8 [9.7]g vs. 48.0 [15.7]g), RVEDVi (69.8 [29.7]mL/m2 vs. 89.1 [21.9]mL/m2 ), and TAE (22.0 [3.2]cm vs. 26.0 [4.7]cm). Significant correlations were found between precontrast RV-T1 and RVEF (beta=-0.48%/msec) and between LV-T1 and LVEF (beta=-0.51%/msec).DATA CONCLUSION: Precontrast RV-T1 is elevated in boys with DMD compared to healthy controls and is negatively correlated with RVEF.LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY: Stage 2.

  View details for DOI 10.1002/jmri.27729

  View details for PubMedID 34037289

• RIGHT VENTRICULAR OUTFLOW TRACT AND PULMONARY ARTERY GEOMETRY IN PATIENTS WITH REPAIRED TETRALOGY OF FALLOT PRIOR TO PULMONARY VALVE REPLACEMENT-CHARACTERIZATION AND LONGITUDINAL ASSOCIATION WITH BIOPROSTHETIC VALVE FUNCTION Arana, V., Chan, F., Schiavone, N., Reddy, S., Hanley, F., Marsden, A., McElhinney, D. ELSEVIER SCIENCE INC. 2021: 1410

  View details for Web of Science ID 000647487501418

• Durability of Pulmonary Valve Replacement with Large Diameter Stented Porcine Bioprostheses. Seminars in thoracic and cardiovascular surgery Maeda, K., Lui, G. K., Zhang, Y., Maskatia, S. A., Romfh, A., Yarlagadda, V. V., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  There is limited information about durability of large diameter porcine bioprostheses implanted for pulmonary valve replacement (PVR). We studied patients who underwent surgical PVR from 2002-2019 with a stented porcine bioprosthetic valve (BPV) with a labeled size ≥27 mm. The primary outcome was freedom from reintervention. During the study period, 203 patients underwent PVR using a porcine BPV ≥27 mm, 94% of whom received a Mosaic valve (Medtronic Inc., Minneapolis, MN). Twenty patients underwent reintervention from 3.4-12.0 years after PVR: 5 surgical and 15 transcatheter PVR procedures. The indication for reintervention was regurgitation in 13 patients, stenosis in 2, mixed disease in 4, and endocarditis in 1. Estimate freedom from reintervention was 97±1% at 5 years and 82±4% at 10 years, and freedom from prosthesis dysfunction (moderate or severe regurgitation and/or a maximum Doppler gradient ≥50 mmHg) over time was 91±2% at 5 years and 74±4% at 10 years. Younger age and smaller true valve diameter were associated with shorter freedom from reintervention, but valve oversizing was not. The durability of large stented porcine bioprostheses in the pulmonary position is generally excellent, particularly in adolescents and adults, similar to various other types of BPV. In the current study, relative valve size was not associated with valve longevity, although the low event-rate in this population was a limiting factor.

  View details for DOI 10.1053/j.semtcvs.2021.03.044

  View details for PubMedID 33971298

• Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals JOURNAL OF INVASIVE CARDIOLOGY Long, Z. B., Adamson, G. T., Peng, L. F., Perry, S. B., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021; 33 (5): E378-E386

  View details for Web of Science ID 000648791300008

• Balloon Angioplasty for Pulmonary Artery Stenosis After Complete Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. The Journal of invasive cardiology Long, Z. B., Adamson, G. T., Peng, L. F., Perry, S. B., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  OBJECTIVES: The objective of this study was to assess procedural outcomes of balloon pulmonary artery (PA) angioplasty procedures after complete repair of tetralogy of Fallot with major aortopulmonary collateral arteries (TOF/MAPCAs).BACKGROUND: Our approach to patients with TOF/MAPCAs emphasizes early complete unifocalization and repair. Major PA reinterventions are relatively uncommon. Balloon PA angioplasty is often used, but the effectiveness of balloon PA angioplasty in this population is unknown.METHODS: The study cohort comprised patients who underwent complete unifocalization and repair of TOF/ MAPCAs at our center between 2002-2018 and underwent balloon PA angioplasty after repair. To assess immediate procedural outcomes, pre- and postintervention PA measurements were compared.RESULTS: We reviewed 134 vessels that were dilated a median of 1.1 years after repair in 60 patients (median 2 PA branches per patient). Treated vessels included 15 central, 64 lobar, and 55 segmental branches. The median PA diameter at the level of stenosis increased from 1.9 mm to 3.3 mm (P<.001), and the median diameter increase was 50%. All but 6 treated vessels were enlarged. The stenosis-distal diameter ratio increased from a median of 64% to 89% (P<.001). The median central PA to aortic systolic pressure ratio was 47% before and 39% after intervention (P<.001).CONCLUSIONS: Balloon PA angioplasty was acutely effective at treating most stenoses of reconstructed PA branches after repair of TOF/MAPCAs. Simple angioplasty can be a useful tool in treating isolated or modest stenoses after unifocalization/PA reconstruction surgery using our approach.

  View details for PubMedID 33908895

• Association between Pre-Albumin and Malnutrition in Children with Advanced Heart Failure Gonzales, S., Schultz, L., Dykes, J. C., Chen, S., Wujcik, K., Kaufman, B., Chen, C., Maeda, K., McElhinney, D. B., Almond, C. ELSEVIER SCIENCE INC. 2021: S203

  View details for Web of Science ID 000631254400464

• Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Kreutzer, J., Armstrong, A. K., Rome, J. J., Zellers, T. M., Balzer, D. T., Zampi, J. D., Cabalka, A. K., Javois, A. J., Turner, D. R., Gray, R. G., Moore, J. W., Weng, S., Jones, T. K., Khan, D. M., Vincent, J. A., Hellenbrand, W. E., Cheatham, J. P., Bergersen, L. J., McElhinney, D. B. 2021

  Abstract

  OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial.BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared.METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing.RESULTS: 167 patients (median age, 19years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both.CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

  View details for DOI 10.1002/ccd.29657

  View details for PubMedID 33780150

• Timing of Transfer and Mortality in Neonates with Hypoplastic Left Heart Syndrome in California. Pediatric cardiology Purkey, N. J., Ma, C., Lee, H. C., Hintz, S. R., Shaw, G. M., McElhinney, D. B., Carmichael, S. L. 2021

  Abstract

  Maternal race/ethnicity is associated with mortality in neonates with hypoplastic left heart syndrome (HLHS). We investigated whether maternal race/ethnicity and other sociodemographic factors affect timing of transfer after birth and whether timing of transfer impacts mortality in infants with HLHS. We linked two statewide databases, the California Perinatal Quality Care Collaborative and records from the Office of Statewide Health Planning and Development, to identify cases of HLHS born between 1/1/06 and 12/31/11. Cases were divided into three groups: birth at destination hospital, transfer on day of life 0-1 ("early transfer"), or transfer on day of life≥2 ("late transfer"). We used log-binomial regression models to estimate relative risks (RR) for timing of transfer and Cox proportional hazard models to estimate hazard ratios (HR) for mortality. We excluded infants who died within 60days of life without intervention from the main analyses of timing of transfer, since intervention may not have been planned in these infants. Of 556 cases, 107 died without intervention (19%) and another 52 (9%) died within 28days. Of the 449 included in analyses of timing of transfer, 28% were born at the destination hospital, 49% were transferred early, and 23% were transferred late. Late transfer was more likely for infants of low birthweight (RR 1.74) and infants born to US-born Hispanic (RR 1.69) and black (RR 2.45) mothers. Low birthweight (HR 1.50), low 5-min Apgar score (HR 4.69), and the presence of other major congenital anomalies (HR 3.41), but not timing of transfer, predicted neonatal mortality. Late transfer was more likely in neonates born to US-born Hispanic and black mothers but was not associated with higher mortality.

  View details for DOI 10.1007/s00246-021-02561-w

  View details for PubMedID 33533967

• Semilunar Valve Interventions for Congenital Heart Disease: JACC State-of-the-Art Review. Journal of the American College of Cardiology Morray, B. H., McElhinney, D. B. 2021; 77 (1): 71–79

  Abstract

  Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.

  View details for DOI 10.1016/j.jacc.2020.10.052

  View details for PubMedID 33413944

• In Vitro Assessment of Right Ventricular Outflow Tract Anatomy and Valve Orientation Effects on Bioprosthetic Pulmonary Valve Hemodynamics. Cardiovascular engineering and technology Schiavone, N. K., Elkins, C. J., McElhinney, D. B., Eaton, J. K., Marsden, A. L. 2021

  Abstract

  The congenital heart defect Tetralogy of Fallot (ToF) affects 1 in 2500 newborns annually in the US and typically requires surgical repair of the right ventricular outflow tract (RVOT) early in life, with variations in surgical technique leading to large disparities in RVOT anatomy among patients. Subsequently, often in adolescence or early adulthood, patients usually require surgical placement of a xenograft or allograft pulmonary valve prosthesis. Valve longevity is highly variable for reasons that remain poorly understood.This work aims to assess the performance of bioprosthetic pulmonary valves in vitro using two 3D printed geometries: an idealized case based on healthy subjects aged 11 to 13 years and a diseased case with a 150% dilation in vessel diameter downstream of the valve. Each geometry was studied with two valve orientations: one with a valve leaflet opening posterior, which is the native pulmonary valve position, and one with a valve leaflet opening anterior.Full three-dimensional, three-component, phase-averaged velocity fields were obtained in the physiological models using 4D flow MRI. Flow features, particularly vortex formation and reversed flow regions, differed significantly between the RVOT geometries and valve orientations. Pronounced asymmetry in streamwise velocity was present in all cases, while the diseased geometry produced additional asymmetry in radial flows. Quantitative integral metrics demonstrated increased secondary flow strength and recirculation in the rotated orientation for the diseased geometry.The compound effects of geometry and orientation on bioprosthetic valve hemodynamics illustrated in this study could have a crucial impact on long-term valve performance.

  View details for DOI 10.1007/s13239-020-00507-6

  View details for PubMedID 33452649

• Comprehensive diagnostic catheterization in children with major aortopulmonary collateral arteries: A review of catheterization technique and anatomic nomenclature. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Perry, S. B., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  Achieving an optimal surgical result in patients with major aortopulmonary collateral arteries (MAPCAs) requires a thorough preoperative evaluation of the anatomy and physiology of the pulmonary circulation. This review provides a detailed description of diagnostic catheterization in patients with MAPCAs, including a summary of catheterization techniques, an overview of commonly used terms, and a review of MAPCA and pulmonary artery angiographic anatomy.

  View details for DOI 10.1002/ccd.30013

  View details for PubMedID 34800077

• A Fetal Risk Stratification Pathway for Neonatal Aortic Coarctation Reduces Medical Exposure. The Journal of pediatrics Maskatia, S. A., Kwiatkowski, D., Bhombal, S., Davis, A. S., McElhinney, D. B., Tacy, T. A., Algaze, C., Blumenfeld, Y., Quirin, A., Punn, R. 2021

  Abstract

  To test the hypothesis that a fetal stratification pathway will effectively discriminate between infants at different levels of risk for surgical coarctation and reduce unnecessary medicalization.We performed a pre-post non-randomized study in which we prospectively assigned fetuses with prenatal concern for coarctation to one of three risk categories and implemented a clinical pathway for postnatal management. Postnatal clinical outcomes were compared with a historical control group that were not triaged based on the pathway.The study cohort included 109 fetuses, 57 treated along the fetal coarctation pathway, and 52 historical controls. Among mild-risk fetuses, 3% underwent surgical coarctation repair (0% of those without additional heart defects), compared with 27% of moderate-risk and 63% of high-risk. Combined fetal aortic, mitral and isthmus z-score best discriminated which patients underwent surgery, AUC=0.78(0.66,0.91). Patients triaged according to the fetal coarctation pathway had reduced delivery location changes (76% vs 55%, p=0.025), and umbilical venous catheter placement (74% vs 51%, p=0.046) compared with historical controls. Trends towards shorter intensive care unit stay, hospital stay and time to enteral feeding did not reach statistical significance.A stratified risk-assignment pathway effectively identifies a group of fetuses with low rate of surgical coarctation, and reduces unnecessary medicalization in infants who do not undergo aortic surgery. Incorporation of novel measurements or imaging techniques may improve specificity of high-risk criteria.

  View details for DOI 10.1016/j.jpeds.2021.06.047

  View details for PubMedID 34181988

• Identification of patients at risk of new onset heart failure: Utilizing a large statewide health information exchange to train and validate a risk prediction model. PloS one Duong, S. Q., Zheng, L., Xia, M., Jin, B., Liu, M., Li, Z., Hao, S., Alfreds, S. T., Sylvester, K. G., Widen, E., Teuteberg, J. J., McElhinney, D. B., Ling, X. B. 2021; 16 (12): e0260885

  Abstract

  BACKGROUND: New-onset heart failure (HF) is associated with poor prognosis and high healthcare utilization. Early identification of patients at increased risk incident-HF may allow for focused allocation of preventative care resources. Health information exchange (HIE) data span the entire spectrum of clinical care, but there are no HIE-based clinical decision support tools for diagnosis of incident-HF. We applied machine-learning methods to model the one-year risk of incident-HF from the Maine statewide-HIE.METHODS AND RESULTS: We included subjects aged ≥ 40 years without prior HF ICD9/10 codes during a three-year period from 2015 to 2018, and incident-HF defined as assignment of two outpatient or one inpatient code in a year. A tree-boosting algorithm was used to model the probability of incident-HF in year two from data collected in year one, and then validated in year three. 5,668 of 521,347 patients (1.09%) developed incident-HF in the validation cohort. In the validation cohort, the model c-statistic was 0.824 and at a clinically predetermined risk threshold, 10% of patients identified by the model developed incident-HF and 29% of all incident-HF cases in the state of Maine were identified.CONCLUSIONS: Utilizing machine learning modeling techniques on passively collected clinical HIE data, we developed and validated an incident-HF prediction tool that performs on par with other models that require proactively collected clinical data. Our algorithm could be integrated into other HIEs to leverage the EMR resources to provide individuals, systems, and payors with a risk stratification tool to allow for targeted resource allocation to reduce incident-HF disease burden on individuals and health care systems.

  View details for DOI 10.1371/journal.pone.0260885

  View details for PubMedID 34890438

• Longevity of large aortic allograft conduits in Tetralogy with major aortopulmonary collaterals. The Annals of thoracic surgery Ma, M. n., Arunamata, A. n., Peng, L. F., Wise-Faberowski, L. n., Hanley, F. L., McElhinney, D. B. 2021

  Abstract

  Appropriate conduit selection for right ventricle (RV) to pulmonary artery (PA) connection has been extensively studied, with older implantation age, pulmonary (v. aortic) homografts, and true-sizing associated with increased longevity. Notably, patients with PA arborization abnormalities (ie MAPCAs) are reported to require earlier and more frequent conduit interventions. We aim to understand the behavior of large diameter aortic homografts in patients with MAPCAs, which are programmatically utilized at our institution.This is a single-center retrospective cohort study including all children < 12 years of age who underwent RV-PA connection using an aortic homograft ≥ 16mm diameter between 2002-2019, with a primary outcome of freedom from any RV-PA reintervention and a secondary outcome of freedom from surgical reintervention. Patients were grouped by absolute and indexed conduit sizes for further analysis.A total of 336 conduits were followed for a median of 3.0 years; transcatheter (n = 30) and/or surgical (n = 35) reintervention was performed on 64 conduits. Estimated freedom from reintervention and surgical replacement was 84% and 90% at 5 years. Younger age and smaller absolute conduit size were associated with earlier reintervention, but conduit z-score (median 3.5) was not assocated with outcome.The programmatic use of oversized aortic homograft RV-PA conduits in the surgical repair of MAPCAs provides a focused experience that demonstrates similar longevity to reported best alternatives. Secondarily, conduit oversizing may improve durability and enables an increased likelihood of non-operative reintervention.

  View details for DOI 10.1016/j.athoracsur.2021.01.054

  View details for PubMedID 33600790

• Mid-Term Outcomes After Unifocalization Guided by Intraoperative Pulmonary Flow Study. World journal for pediatric & congenital heart surgery Goodman, A., Ma, M., Zhang, Y., Ryan, K. R., Jahadi, O., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2021; 12 (1): 76–83

  Abstract

  BACKGROUND: Repair of tetralogy of Fallot (TOF) with major aortopulmonary collateral arteries (MAPCAs) requires unifocalization of pulmonary circulation, intracardiac repair with the closure of the ventricular septal defect, and placement of a right ventricle (RV) to pulmonary artery (PA) conduit. The decision to perform complete repair is sometimes aided by an intraoperative flow study to estimate the total resistance of the reconstructed pulmonary circulation.METHODS: We reviewed patients who underwent unifocalization and PA reconstruction for TOF/MAPCAs to evaluate acute and mid-term outcomes after repair with and without flow studies and to characterize the relationship between PA pressure during the flow study and postrepair RV pressure.RESULTS: Among 579 patients who underwent unifocalization and PA reconstruction for TOF/MAPCAs, 99 (17%) had an intraoperative flow study during one (n = 91) or more (n = 8) operations to determine the suitability for a complete repair. There was a reasonably good correlation between mean PA pressure at 3 L/min/m2 during the flow study and postrepair RV pressure and RV:aortic pressure ratio. Acute and mid-term outcomes (median: 3.8 years) after complete repair in the flow study patients (n = 78) did not differ significantly from those in whom the flow study was not performed (n = 444). Furthermore, prior failed flow study was not associated with differences in outcome after subsequent intracardiac repair.CONCLUSIONS: The intraoperative flow study remains a useful adjunct for determining the suitability for complete repair in a subset of patients undergoing surgery for TOF/MAPCAs, as it is reasonably accurate for estimating postoperative PA pressure and serves as a reliable guide for the feasibility of single-stage complete repair.

  View details for DOI 10.1177/2150135120964427

  View details for PubMedID 33407027

• Alternative to heart-lung transplantation for end-stage tetralogy of Fallot with major aortopulmonary collaterals: Simultaneous heart transplantation and pulmonary artery reconstruction. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Adamson, G. T., Profita, E. L., Quinonez, Z. A., McElhinney, D. B., Rosenthal, D. N., Ma, M. n. 2021

  View details for DOI 10.1016/j.healun.2021.02.003

  View details for PubMedID 33674153

• Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis. Journal of the American College of Cardiology Shahanavaz, S., Zahn, E. M., Levi, D. S., Aboulhousn, J. A., Hascoet, S., Qureshi, A. M., Porras, D., Morgan, G. J., Bauser Heaton, H., Martin, M. H., Keeshan, B., Asnes, J. D., Kenny, D., Ringewald, J. M., Zablah, J. E., Ivy, M., Morray, B. H., Torres, A. J., Berman, D. P., Gillespie, M. J., Chaszczewski, K., Zampi, J. D., Walsh, K. P., Julien, P., Goldstein, B. H., Sathanandam, S. K., Karsenty, C., Balzer, D. T., McElhinney, D. B. 2020; 76 (24): 2847–58

  Abstract

  BACKGROUND: There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve.OBJECTIVES: This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve.METHODS: Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy.RESULTS: Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40mmHg. During limited follow-up (n=349; median: 12months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR.CONCLUSIONS: Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all typesofRVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

  View details for DOI 10.1016/j.jacc.2020.10.041

  View details for PubMedID 33303074

• Maternal metabolic profiling to assess fetal gestational age and predict preterm delivery: a two-centre retrospective cohort study in the US. BMJ open Sylvester, K. G., Hao, S., You, J., Zheng, L., Tian, L., Yao, X., Mo, L., Ladella, S., Wong, R. J., Shaw, G. M., Stevenson, D. K., Cohen, H. J., Whitin, J. C., McElhinney, D. B., Ling, X. B. 2020; 10 (12): e040647

  Abstract

  OBJECTIVES: The aim of this study was to develop a single blood test that could determine gestational age and estimate the risk of preterm birth by measuring serum metabolites. We hypothesised that serial metabolic modelling of serum analytes throughout pregnancy could be used to describe fetal gestational age and project preterm birth with a high degree of precision.STUDY DESIGN: A retrospective cohort study.SETTING: Two medical centres from the USA.PARTICIPANTS: Thirty-six patients (20 full-term, 16 preterm) enrolled at Stanford University were used to develop gestational age and preterm birth risk algorithms, 22 patients (9 full-term, 13 preterm) enrolled at the University of Alabama were used to validate the algorithms.OUTCOME MEASURES: Maternal blood was collected serially throughout pregnancy. Metabolic datasets were generated using mass spectrometry.RESULTS: A model to determine gestational age was developed (R2=0.98) and validated (R2=0.81). 66.7% of the estimates fell within ±1week of ultrasound results during model validation. Significant disruptions from full-term pregnancy metabolic patterns were observed in preterm pregnancies (R2=-0.68). A separate algorithm to predict preterm birth was developed using a set of 10 metabolic pathways that resulted in an area under the curve of 0.96 and 0.92, a sensitivity of 0.88 and 0.86, and a specificity of 0.96 and 0.92 during development and validation testing, respectively.CONCLUSIONS: In this study, metabolic profiling was used to develop and test a model for determining gestational age during full-term pregnancy progression, and to determine risk of preterm birth. With additional patient validation studies, these algorithms may be used to identify at-risk pregnancies prompting alterations in clinical care, and to gain biological insights into the pathophysiology of preterm birth. Metabolic pathway-based pregnancy modelling is a novel modality for investigation and clinical application development.

  View details for DOI 10.1136/bmjopen-2020-040647

  View details for PubMedID 33268420

• Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study. Journal of the American Heart Association Freud, L. R., McElhinney, D. B., Kalish, B. T., Escobar-Diaz, M. C., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Selamet Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., Tworetzky, W. 2020: e016684

  Abstract

  Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

  View details for DOI 10.1161/JAHA.120.016684

  View details for PubMedID 33076749

• Management and Outcomes of Transvenous Pacing Leads in PatientsUndergoing Transcatheter Tricuspid Valve Replacement. JACC. Cardiovascular interventions Anderson, J. H., McElhinney, D. B., Aboulhosn, J., Zhang, Y., Ribichini, F., Eicken, A., Whisenant, B., Jones, T., Kornowski, R., Dvir, D., Cabalka, A. K., VIVID Registry 2020

  Abstract

  OBJECTIVES: The aim of this study was to determine the prevalence of pacemaker lead-related complications following transcatheter tricuspid valve replacement (TTVR).BACKGROUND: The rate of permanent pacemaker implantation following tricuspid valve (TV) surgery is high, and many patients have transvenous leads. The feasibility, safety, and outcomes of subsequently performing TTVR in the setting of transvenous pacemaker leads have not been established.METHODS: The VIVID (Valve-in-Valve International Database) registry was used to review 329 patients who underwent TTVR following TV repair or replacement. Patients were subdivided into 3 cohorts for intergroup comparisons: no lead, epicardial lead, and transvenous lead (entrapped or not entrapped during the TTVR procedure).RESULTS: Of 329 patients who underwent TTVR, 128 (39%) had prior pacing systems in place, 70 with epicardial and 58 with transvenous leads. A total of 31 patients had leads passing through the TV. Three patients had the right ventricular (RV) lead extracted prior to TTVR. The remaining 28 patients had the RV lead entrapped between the transcatheter TV implant and the surgical valve (n=22) or the repaired TV (n=6). One patient had displacement of the RV lead during the procedure, and 2 experienced lead failure during follow-up. Overall, there was no significant difference in the cumulative incidences of competing outcomes (death, TV reintervention, TV dysfunction) between patients with and those without pacing leads or entrapped RV leads.CONCLUSIONS: TTVR in the setting of trans-TV pacemaker leads without lead extraction or re-replacement can be performed safely with a low risk for complications, offering an alternative to surgical TV replacement.

  View details for DOI 10.1016/j.jcin.2020.04.054

  View details for PubMedID 32800497

• Outcomes After Transcatheter Reintervention for Dysfunction of a Previously Implanted Transcatheter Pulmonary Valve. JACC. Cardiovascular interventions Shahanavaz, S., Berger, F., Jones, T. K., Kreutzer, J., Vincent, J. A., Eicken, A., Bergersen, L., Rome, J. J., Zahn, E., Sondergaard, L., Cheatham, J. P., Weng, S., Balzer, D., McElhinney, D. 2020; 13 (13): 1529–40

  Abstract

  OBJECTIVES: The aim of this analysis was to evaluate outcomes following transcatheter reintervention for degenerated transcatheter pulmonary valves (TPVs).BACKGROUND: TPV replacement (TPVR) with the Melody valve demonstrated sustained relief of right ventricular outflow tract (RVOT) obstruction and pulmonary regurgitation.METHODS: All patients who underwent TPVR with a Melody valve as part of 3 Medtronic-sponsored prospective multicenter studies were included. Transcatheter reinterventions included balloon dilation of the previously implanted Melody valve, placement of a bare-metal stent within the implanted TPV, or placement of a new TPV in the RVOT (TPV-in-TPV). Indications for reintervention, decisions to reintervene, and the method of reintervention were at physician discretion. All patients provided written informed consent to participate in the trials, and each trial was approved by local or central Institutional Review Boards or ethics committees at participating sites.RESULTS: A total of 309 patients who underwent TPVR were discharged from the implantation hospitalization with Melody valves in place. Transcatheter reintervention on the TPV was performed in 46 patients. The first transcatheter reintervention consisted of TPV-in-TPV in 28 patients (median 6.9 years [quartile 1 to quartile 3: 5.2 to 7.8 years] after TPVR), simple balloon dilation of the implanted Melody valve in 17 (median 4.9 years [quartile 1 to quartile 3: 4.0 to 6.0 years] after TPVR), and bare-metal stent placement alone in 1 (4.4 years after TPVR). There were no major procedural complications. Overall, 4-year freedom from explant and from any later RVOT reintervention after the first reintervention were 83% and 60%, respectively. Freedom from repeat RVOT reintervention was longer in patients undergoing TPV-in-TPV than balloon dilation (71% vs. 46% at 4 years; p=0.027).CONCLUSIONS: TPV-in-TPV can be an effective and durable treatment for Melody valve dysfunction. Although balloondilation of the Melody valve was also acutely effective at reducing RVOT obstruction, the durability of this therapywaslimited in this cohort compared with TPV-in-TPV.

  View details for DOI 10.1016/j.jcin.2020.03.035

  View details for PubMedID 32646693

• Association between Pre-Albumin and Malnutrition in Children with Advanced Heart Failure Gonzales, S., Schultz, L., Dykes, J., Chen, S., Wujcik, K., Kaufman, B., Chen, C., Maeda, K., McElhinney, D. B., Almond, C. ELSEVIER SCIENCE INC. 2020: S452

  View details for Web of Science ID 000522637203133

• Impact of Cardiac Resynchronization Therapy on Heart Transplant-Free Survival in Pediatric and Congenital Heart Disease Patients. Circulation. Arrhythmia and electrophysiology Chubb, H., Rosenthal, D. N., Almond, C. S., Ceresnak, S. R., Motonaga, K. S., Arunamata, A. A., Long, J., Trela, A. V., Hanisch, D., McElhinney, D. B., Dubin, A. M. 2020

  Abstract

  Background - Cardiac resynchronization therapy (CRT) studies in pediatric and/or congenital heart disease (CHD) patients have shown an improvement in ejection fraction and heart failure symptoms. However, a survival benefit of CRT in this population has not been established. This study aimed to evaluate the impact of CRT upon heart transplant-free survival in pediatric and CHD patients, using a propensity score-matched analysis. Methods - This single-center study compared CRT patients (implant date 2004-2017) and controls, matched by 1:1 propensity-score matching (PSM) using 21 comprehensive baseline indices for risk stratification. CRT patients were <21 years or had CHD; had systemic ventricular ejection fraction <;45%; symptomatic heart failure; and had significant electrical dyssynchrony, all prior to CRT implant. Controls were screened from non-selective imaging and ECG databases. Controls were retrospectively enrolled when they achieved the same inclusion criteria at an outpatient clinical encounter, within the same time period. Results - Of 133 patients who received CRT during the study period, 84 met all study inclusion criteria. 133 controls met all criteria at an outpatient encounter. Following PSM, 63 matched CRT-control pairs were identified with no significant difference between groups across all baseline indices. Heart transplant or death occurred in 12 (19%) PSM-CRT subjects and 37 (59%) PSM-controls with a median follow-up of 2.7 years (quartiles 0.8-6.1 years). CRT was associated with markedly reduced risk of heart transplant or death (hazard ratio 0.24 [95% CI 0.12-0.46], p<0.001). There was no CRT procedural mortality and one system infection at 54 months post-implant. Conclusions - In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplant-free survival.

  View details for DOI 10.1161/CIRCEP.119.007925

  View details for PubMedID 32202126

• Changes in pregnancy-related serum biomarkers early in gestation are associated with later development of preeclampsia. PloS one Hao, S. n., You, J. n., Chen, L. n., Zhao, H. n., Huang, Y. n., Zheng, L. n., Tian, L. n., Maric, I. n., Liu, X. n., Li, T. n., Bianco, Y. K., Winn, V. D., Aghaeepour, N. n., Gaudilliere, B. n., Angst, M. S., Zhou, X. n., Li, Y. M., Mo, L. n., Wong, R. J., Shaw, G. M., Stevenson, D. K., Cohen, H. J., Mcelhinney, D. B., Sylvester, K. G., Ling, X. B. 2020; 15 (3): e0230000

  Abstract

  Placental protein expression plays a crucial role during pregnancy. We hypothesized that: (1) circulating levels of pregnancy-associated, placenta-related proteins throughout gestation reflect the temporal progression of the uncomplicated, full-term pregnancy, and can effectively estimate gestational ages (GAs); and (2) preeclampsia (PE) is associated with disruptions in these protein levels early in gestation; and can identify impending PE. We also compared gestational profiles of proteins in the human and mouse, using pregnant heme oxygenase-1 (HO-1) heterozygote (Het) mice, a mouse model reflecting PE-like symptoms.Serum levels of placenta-related proteins-leptin (LEP), chorionic somatomammotropin hormone like 1 (CSHL1), elabela (ELA), activin A, soluble fms-like tyrosine kinase 1 (sFlt-1), and placental growth factor (PlGF)-were quantified by ELISA in blood serially collected throughout human pregnancies (20 normal subjects with 66 samples, and 20 subjects who developed PE with 61 samples). Multivariate analysis was performed to estimate the GA in normal pregnancy. Mean-squared errors of GA estimations were used to identify impending PE. The human protein profiles were then compared with those in the pregnant HO-1 Het mice.An elastic net-based gestational dating model was developed (R2 = 0.76) and validated (R2 = 0.61) using serum levels of the 6 proteins measured at various GAs from women with normal uncomplicated pregnancies. In women who developed PE, the model was not (R2 = -0.17) associated with GA. Deviations from the model estimations were observed in women who developed PE (P = 0.01). The model developed with 5 proteins (ELA excluded) performed similarly from sera from normal human (R2 = 0.68) and WT mouse (R2 = 0.85) pregnancies. Disruptions of this model were observed in both human PE-associated (R2 = 0.27) and mouse HO-1 Het (R2 = 0.30) pregnancies. LEP outperformed sFlt-1 and PlGF in differentiating impending PE at early human and late mouse GAs.Serum placenta-related protein profiles are temporally regulated throughout normal pregnancies and significantly disrupted in women who develop PE. LEP changes earlier than the well-established biomarkers (sFlt-1 and PlGF). There may be evidence of a causative action of HO-1 deficiency in LEP upregulation in a PE-like murine model.

  View details for DOI 10.1371/journal.pone.0230000

  View details for PubMedID 32126118

• Unifocalization and pulmonary artery reconstruction in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery before referral. The Journal of thoracic and cardiovascular surgery Ma, M. n., Zhang, Y. n., Wise-Faberowski, L. n., Lin, A. n., Asija, R. n., Hanley, F. L., McElhinney, D. B. 2020

  Abstract

  The study objective was to characterize and analyze outcomes in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who had undergone surgery elsewhere before referral (prereferral surgery).Patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery between 2001 and 2019 at our center were reviewed. Prereferral surgery and unoperated patients were compared, as were subsets of prereferral surgery patients who had undergone different types of prior procedures. Primary outcomes included complete repair with survival to 6 months, death, and perioperative metrics.Of 576 patients studied, 200 (35%) had undergone a wide range and number of prior operations elsewhere, including 92 who had pulmonary blood supply through a shunt and 108 who had a right ventricle pulmonary artery connection. Patients who underwent prereferral surgery with an existing right ventricle pulmonary artery connection had undergone more prereferral surgery procedures than those with a shunt and were more likely to have a right ventricle outflow tract pseudoaneurysm or pulmonary artery stent (all P < .001) at the time of referral. The cumulative incidences of complete repair and death were similar regardless of prereferral surgery status, but the cumulative incidence of complete repair with 6-month survival was higher (P = .002) and of death lower (P = .18) in patients who had prior right ventricle pulmonary artery connection compared with those who had received a prior shunt only.Our comprehensive management strategy for tetralogy of Fallot and major aortopulmonary collateral arteries can be applied with excellent procedural results in both unoperated patients and those who have undergone multiple and varied procedures elsewhere.

  View details for DOI 10.1016/j.jtcvs.2020.03.062

  View details for PubMedID 32444187

• Multicentre validation of a computer-based tool for differentiation of acute Kawasaki disease from clinically similar febrile illnesses. Archives of disease in childhood Hao, S. n., Ling, X. B., Kanegaye, J. T., Bainto, E. n., Dominguez, S. R., Heizer, H. n., Jone, P. N., Anderson, M. S., Jaggi, P. n., Baker, A. n., Son, M. B., Newberger, J. W., Ashouri, N. n., McElhinney, D. B., Burns, J. C., Whitin, J. C., Cohen, H. J., Tremoulet, A. H. 2020

  Abstract

  The clinical features of Kawasaki disease (KD) overlap with those of other paediatric febrile illnesses. A missed or delayed diagnosis increases the risk of coronary artery damage. Our computer algorithm for KD and febrile illness differentiation had a sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 94.8%, 70.8%, 93.7% and 98.3%, respectively, in a single-centre validation study. We sought to determine the performance of this algorithm with febrile children from multiple institutions across the USA.We used our previously published 18-variable panel that includes illness day, the five KD clinical criteria and readily available laboratory values. We applied this two-step algorithm using a linear discriminant analysis-based clinical model followed by a random forest-based algorithm to a cohort of 1059 acute KD and 282 febrile control patients from five children's hospitals across the USA.The algorithm correctly classified 970 of 1059 patients with KD and 163 of 282 febrile controls resulting in a sensitivity of 91.6%, specificity of 57.8% and PPV and NPV of 95.4% and 93.1%, respectively. The algorithm also correctly identified 218 of the 232 KD patients (94.0%) with abnormal echocardiograms.The expectation is that the predictive accuracy of the algorithm will be reduced in a real-world setting in which patients with KD are rare and febrile controls are common. However, the results of the current analysis suggest that this algorithm warrants a prospective, multicentre study to evaluate its potential utility as a physician support tool.

  View details for DOI 10.1136/archdischild-2019-317980

  View details for PubMedID 32139365

• SCAI position statement on adult congenital cardiac interventional training, competencies and organizational recommendations. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Aboulhosn, J. A., Hijazi, Z. M., Kavinsky, C. J., McElhinney, D. B., Asgar, A. W., Benson, L. N., Daniels, C. J., Ghobrial, J. n., Horlick, E. n., Ing, F. F., Inglessis, I. n., Kay, J. n., Levi, D. S. 2020

  View details for DOI 10.1002/ccd.28885

  View details for PubMedID 32272495

• Prevention and management of endocarditis after transcatheter pulmonary valve replacement: current status and future prospects. Expert review of medical devices McElhinney, D. B. 2020: 1–8

  Abstract

  Introduction: Transcatheter pulmonary valve replacement (TPVR) has become an important tool in the management of congenital heart disease with abnormalities of the right ventricular outflow tract. Endocarditis is one of the most serious adverse long-term outcomes and among the leading causes of death in patients with congenital heart disease and after (TPVR). Areas covered: This review discusses the current state knowledge about the risk factors for and outcomes of endocarditis after transcatheter pulmonary valve replacement in patients with congenital and acquired heart disease. It also addresses practical measures for mitigating endocarditis risk, as well as diagnosing and managing endocarditis when it does occur. Expert opinion: With increasing understanding of the risk factors for and management and outcomes of endocarditis in patients who have undergone TPVR, we continue to learn how to utilize TPVR most effectively in this complex population of patients.

  View details for DOI 10.1080/17434440.2021.1857728

  View details for PubMedID 33246368

• Kinetics of SARS-CoV-2 positivity of infected and recovered patients from a single center. Scientific reports Huang, J. n., Zheng, L. n., Li, Z. n., Hao, S. n., Ye, F. n., Chen, J. n., Gans, H. A., Yao, X. n., Liao, J. n., Wang, S. n., Zeng, M. n., Qiu, L. n., Li, C. n., Whitin, J. C., Tian, L. n., Chubb, H. n., Hwa, K. Y., Ceresnak, S. R., Zhang, W. n., Lu, Y. n., Maldonado, Y. A., McElhinney, D. B., Sylvester, K. G., Cohen, H. J., Liu, L. n., Ling, X. B. 2020; 10 (1): 18629

  Abstract

  Recurrence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive detection in infected but recovered individuals has been reported. Patients who have recovered from coronavirus disease 2019 (COVID-19) could profoundly impact the health care system. We sought to define the kinetics and relevance of PCR-positive recurrence during recovery from acute COVID-19 to better understand risks for prolonged infectivity and reinfection. A series of 414 patients with confirmed SARS-Cov-2 infection, at The Second Affiliated Hospital of Southern University of Science and Technology in Shenzhen, China from January 11 to April 23, 2020. Statistical analyses were performed of the clinical, laboratory, radiologic image, medical treatment, and clinical course of admission/quarantine/readmission data, and a recurrence predictive algorithm was developed. 16.7% recovered patients with PCR positive recurring one to three times, despite being in strict quarantine. Younger patients with mild pulmonary respiratory syndrome had higher risk of PCR positivity recurrence. The recurrence prediction model had an area under the ROC curve of 0.786. This case series provides characteristics of patients with recurrent SARS-CoV-2 positivity. Use of a prediction algorithm may identify patients at high risk of recurrent SARS-CoV-2 positivity and help to establish protocols for health policy.

  View details for DOI 10.1038/s41598-020-75629-x

  View details for PubMedID 33122706

• Unilateral branch pulmonary artery origin from a solitary arterial trunk with major aortopulmonary collaterals to the contralateral lung: anatomic and developmental considerations. Seminars in thoracic and cardiovascular surgery Adamson, G. T., Houyel, L. n., McElhinney, D. B., Maskatia, S. A., Ma, M. n., Hanley, F. L., Lopez, L. n. 2020

  Abstract

  In both truncus arteriosus communis (TAC) and tetralogy of Fallot (TOF), there is a rare phenotype that includes a single branch pulmonary artery (PA) arising from a solitary great artery and major aortopulmonary collaterals (MAPCAs) supplying the contralateral lung. We describe the intracardiac and great vessel anatomy of infants with this phenotype, consider rationale for classifying patients as TOF vs. TAC, and describe surgical outcomes. Our institution's surgical database was reviewed for patients with a single branch PA from a solitary arterial trunk and contralateral MAPCAs from 2007 to 2019. Demographic, imaging, and surgical data were collected and described. All 11 patients underwent complete repair with a median right ventricular to aortic pressure ratio of 0.36 (range 0.26-0.50). At 0.1-9.1 years after repair (median 0.8 years) there was approximately balanced left-right lung perfusion (median 52% to the right lung, range 34-74%). The MAPCA lungs exemplified the full spectrum of PA and MAPCA anatomy, from absent intrapericardial PAs with all single supply MAPCAs to a normally arborizing PA with all dual supply MAPCAs and present pulmonary valve leaflet tissue. All patients had a systemic semilunar valve with 3 thin and similarly sized leaflets and fibrous continuity with the tricuspid valve, and all had coronary origins and outflow tract morphology more consistent with TOF. It is appropriate to classify all patients with a single anomalous PA from a solitary arterial trunk and MAPCAs to the contralateral lung as TOF rather than TAC Type A3. All variants were amenable to surgical repair.

  View details for DOI 10.1053/j.semtcvs.2020.11.009

  View details for PubMedID 33181302

• Identification of elders at higher risk for fall with statewide electronic health records and a machine learning algorithm. International journal of medical informatics Ye, C. n., Li, J. n., Hao, S. n., Liu, M. n., Jin, H. n., Zheng, L. n., Xia, M. n., Jin, B. n., Zhu, C. n., Alfreds, S. T., Stearns, F. n., Kanov, L. n., Sylvester, K. G., Widen, E. n., McElhinney, D. n., Ling, X. B. 2020; 137: 104105

  Abstract

  Predicting the risk of falls in advance can benefit the quality of care and potentially reduce mortality and morbidity in the older population. The aim of this study was to construct and validate an electronic health record-based fall risk predictive tool to identify elders at a higher risk of falls.The one-year fall prediction model was developed using the machine-learning-based algorithm, XGBoost, and tested on an independent validation cohort. The data were collected from electronic health records (EHR) of Maine from 2016 to 2018, comprising 265,225 older patients (≥65 years of age).This model attained a validated C-statistic of 0.807, where 50 % of the identified high-risk true positives were confirmed to fall during the first 94 days of next year. The model also captured in advance 58.01 % and 54.93 % of falls that happened within the first 30 and 30-60 days of next year. The identified high-risk patients of fall showed conditions of severe disease comorbidities, an enrichment of fall-increasing cardiovascular and mental medication prescriptions and increased historical clinical utilization, revealing the complexity of the underlying fall etiology. The XGBoost algorithm captured 157 impactful predictors into the final predictive model, where cognitive disorders, abnormalities of gait and balance, Parkinson's disease, fall history and osteoporosis were identified as the top-5 strongest predictors of the future fall event.By using the EHR data, this risk assessment tool attained an improved discriminative ability and can be immediately deployed in the health system to provide automatic early warnings to older adults with increased fall risk and identify their personalized risk factors to facilitate customized fall interventions.

  View details for DOI 10.1016/j.ijmedinf.2020.104105

  View details for PubMedID 32193089

• Transfusion Outcomes in Patients Undergoing Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. World journal for pediatric & congenital heart surgery Wise-Faberowski, L. n., Irvin, M. n., Quinonez, Z. A., Long, J. n., Asija, R. n., Margetson, T. D., Hanley, F. L., McElhinney, D. B. 2020; 11 (2): 159–65

  Abstract

  Surgical repair of tetralogy of Fallot and major aortopulmonary collaterals (TOF/MAPCAs) involves unifocalization of MAPCAs and reconstruction of the pulmonary arterial circulation. Surgical and cardiopulmonary bypass (CPB) times are long and suture lines are extensive. Maintaining patency of the newly anastomosed vessels while achieving hemostasis is important, and assessment of transfusion practices is critical to successful outcomes.Clinical, surgical, and transfusion data in patients with TOF/MAPCAs repaired at our institution (2013-2018) were reviewed. Types and volumes of blood products used in the perioperative period, in addition to the use of antifibrinolytics and/or procoagulants (factor VIII inhibitor bypassing activity [FEIBA]; anti-inhibitor coagulant complex), were assessed. Outcome measures included days on mechanical ventilation (DOMV), postoperative intensive care unit and hospital length of stay (LoS), and incidence of thrombosis.Perioperative transfusion data from 279 patients were analyzed. Surgical (879 ± 175 minutes vs 684 ± 257 minutes) and CPB times (376 ± 124 minutes vs 234 ± 122 minutes) were longer in patients who received FEIBA than those who did not. Although the indexed volume of packed red blood cells (128.4 ± 82.2 mL/kg) and fresh frozen plasma (64.2 ± 41.1 mL/kg) was similar in patients who did and did not receive FEIBA, the amounts of cryoprecipitate (5.5 ± 5.2 mL/kg vs 5.8 ± 4.8 mL/kg) and platelets (19.5 ± 20.7 mL/kg vs 20.8 ± 13 mL/kg) transfused were more in those who did receive FEIBA.Perioperative transfusion is an important component in the overall surgical and anesthetic management of patients with TOF/MAPCAs. The intraoperative use of FEIBA was not associated with a decrease in the amount of blood products transfused, DOMV, or LoS or with an increase in thrombotic complications.

  View details for DOI 10.1177/2150135119892192

  View details for PubMedID 32093560

• Outcomes in Patients with Alagille Syndrome and Complex Pulmonary Artery Disease. The Journal of pediatrics Luong, R. n., Feinstein, J. A., Ma, M. n., Ebel, N. H., Wise-Faberowski, L. n., Zhang, Y. n., Peng, L. F., Yarlagadda, V. V., Shek, J. n., Hanley, F. L., McElhinney, D. B. 2020

  Abstract

  To assess outcomes in a large cohort of patients with Alagille Syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex PA disease.Patients with ALGS who underwent PA reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch PA stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs RESULTS: Fifty-one patients with ALGS underwent PA surgery at our center: 22 with severe branch PA stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) had a complete repair at the first surgery, compared with 8 (89%) and 19 (86%) with TOF without MAPCAs and isolated branch PA stenosis, respectively. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median PA:aortic systolic pressure ratio of 0.38. Nine patients (18%), 8 with isolated branch PA stenosis, underwent liver transplantation.Most patients with ALGS and complex PA disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcome, with higher mortality and more frequent PA interventions compared with patients with TOF without MAPCAs or isolated branch PA stenosis. Complex PA disease is not a contraindication to liver transplantation in patients with ALGS.

  View details for DOI 10.1016/j.jpeds.2020.09.053

  View details for PubMedID 32980376

• Angiographic Anatomy of Major Aortopulmonary Collateral Arteries and Association With Early Surgical Outcomes in Tetralogy of Fallot. Journal of the American Heart Association Adamson, G. T., McElhinney, D. B., Zhang, Y. n., Feinstein, J. A., Peng, L. F., Ma, M. n., Algaze, C. A., Hanley, F. L., Perry, S. B. 2020: e017981

  Abstract

  Background Due in part to the heterogeneity of the pulmonary circulation in patients with tetralogy of Fallot and major aortopulmonary collateral arteries (MAPCAs), research on this condition has focused on relatively basic anatomic characteristics. We aimed to detail pulmonary artery (PA) and MAPCA anatomy in a large group of infants, assess relationships between anatomy and early surgical outcomes, and consider systems for classifying MAPCAs. Methods and Results All infants ( <1 year of age) undergoing first cardiac surgery for tetralogy of Fallot/MAPCAs from 2001 to 2019 at Stanford University were identified. Preoperative angiograms delineating supply to all 18 pulmonary segments were reviewed for details of each MAPCA and the arborization and size of central PAs. We studied 276 patients with 1068 MAPCAs and the following PA patterns: 152 (55%) incompletely arborizing PAs, 48 (17%) normally arborizing PAs, 45 (16%) absent PAs, and 31 (11%) unilateral MAPCAs. There was extensive anatomic variability, but no difference in early outcomes according to PA arborization or the predominance of PAs or MAPCAs. Patients with low total MAPCA and/or PA cross-sectional area were less likely to undergo complete repair. Conclusions MAPCA anatomy is highly variable and essentially unique for each patient. Though each pulmonary segment can be supplied by a MAPCA, central PA, or both, all anatomic combinations are similarly conducive to a good repair. Total cross-sectional area of central PA and MAPCA material is an important driver of outcome. We elucidate a number of novel associations between anatomic features, but the extreme variability of the pulmonary circulation makes a granular tetralogy of Fallot/MAPCA classification system unrealistic.

  View details for DOI 10.1161/JAHA.120.017981

  View details for PubMedID 33283588

• High-throughput quantitation of serological ceramides/dihydroceramides by LC/MS/MS: Pregnancy baseline biomarkers and potential metabolic messengers. Journal of pharmaceutical and biomedical analysis Huang, Q. n., Hao, S. n., Yao, X. n., You, J. n., Li, X. n., Lai, D. n., Han, C. n., Schilling, J. n., Hwa, K. Y., Thyparambil, S. n., Whitin, J. n., Cohen, H. J., Chubb, H. n., Ceresnak, S. R., McElhinney, D. B., Wong, R. J., Shaw, G. M., Stevenson, D. K., Sylvester, K. G., Ling, X. B. 2020; 192: 113639

  Abstract

  Ceramides and dihydroceramides are sphingolipids that present in abundance at the cellular membrane of eukaryotes. Although their metabolic dysregulation has been implicated in many diseases, our knowledge about circulating ceramide changes during the pregnancy remains limited. In this study, we present the development and validation of a high-throughput liquid chromatography-tandem mass spectrometric method for simultaneous quantification of 16 ceramides and 10 dihydroceramides in human serum within 5 min. by using stable isotope-labeled ceramides as internal standards. This method employs a protein precipitation method for high throughput sample preparation, reverse phase isocratic elusion for chromatographic separation, and Multiple Reaction Monitoring for mass spectrometric detection. To qualify for clinical applications, our assay has been validated against the FDA guidelines for Lower Limit of Quantitation (1 nM), linearity (R2>0.99), precision (imprecision<15 %), accuracy (inaccuracy<15 %), extraction recovery (>90 %), stability (>85 %), and carryover (<0.01 %). With enhanced sensitivity and specificity from this method, we have, for the first time, determined the serological levels of ceramides and dihydroceramides to reveal unique temporal gestational patterns. Our approach could have value in providing insights into disorders of pregnancy.

  View details for DOI 10.1016/j.jpba.2020.113639

  View details for PubMedID 33017796

• Development of an early-warning system for high-risk patients for suicide attempt using deep learning and electronic health records. Translational psychiatry Zheng, L. n., Wang, O. n., Hao, S. n., Ye, C. n., Liu, M. n., Xia, M. n., Sabo, A. N., Markovic, L. n., Stearns, F. n., Kanov, L. n., Sylvester, K. G., Widen, E. n., McElhinney, D. B., Zhang, W. n., Liao, J. n., Ling, X. B. 2020; 10 (1): 72

  Abstract

  Suicide is the tenth leading cause of death in the United States (US). An early-warning system (EWS) for suicide attempt could prove valuable for identifying those at risk of suicide attempts, and analyzing the contribution of repeated attempts to the risk of eventual death by suicide. In this study we sought to develop an EWS for high-risk suicide attempt patients through the development of a population-based risk stratification surveillance system. Advanced machine-learning algorithms and deep neural networks were utilized to build models with the data from electronic health records (EHRs). A final risk score was calculated for each individual and calibrated to indicate the probability of a suicide attempt in the following 1-year time period. Risk scores were subjected to individual-level analysis in order to aid in the interpretation of the results for health-care providers managing the at-risk cohorts. The 1-year suicide attempt risk model attained an area under the curve (AUC ROC) of 0.792 and 0.769 in the retrospective and prospective cohorts, respectively. The suicide attempt rate in the "very high risk" category was 60 times greater than the population baseline when tested in the prospective cohorts. Mental health disorders including depression, bipolar disorders and anxiety, along with substance abuse, impulse control disorders, clinical utilization indicators, and socioeconomic determinants were recognized as significant features associated with incident suicide attempt.

  View details for DOI 10.1038/s41398-020-0684-2

  View details for PubMedID 32080165

• Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair CONGENITAL HEART DISEASE Annavajjhala, V., Punn, R., Tacy, T. A., Hanley, F. L., McElhinney, D. B. 2019; 14 (5): 691–99

  View details for DOI 10.1111/chd.12772

  View details for Web of Science ID 000490040700002

• Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals ANNALS OF THORACIC SURGERY Bauser-Heaton, H., Ma, M., McElhinney, D. B., Goodyer, W. R., Zhang, Y., Chan, F. P., Asija, R., Shek, J., Wise-Faberowski, L., Hanley, F. L. 2019; 108 (3): 820–27

  View details for DOI 10.1016/j.athoracsur.2019.03.022

  View details for Web of Science ID 000482187400054

• Secondary repair of incompetent pulmonary valves after previous surgery or intervention: Patient selection and outcomes. The Journal of thoracic and cardiovascular surgery Adamson, G. T., McElhinney, D. B., Lui, G., Meadows, A. K., Rigdon, J., Hanley, F. L., Maskatia, S. A. 2019

  Abstract

  OBJECTIVES: Pulmonary valve (PV) regurgitation (PR) is common after intervention for a hypoplastic right ventricular outflow tract. Secondary PV repair is an alternative to replacement (PVR), but selection criteria are not established. We sought to elucidate preoperative variables associated with successful PV repair and to compare outcomes between repair and PVR.METHODS: Patients who underwent surgery for secondary PR from 2010 to 2017 by a single surgeon were studied. The PV annulus and leaflets were measured on the preoperative echocardiogram and magnetic resonance images, and the primary predictor variable was leaflet area indexed to ideal PV annulus area (iPLA) by magnetic resonance imaging. PV repair and PVR groups were compared using multivariable logistic regression, and with a conditional inference tree. Freedom from PV dysfunction and from reintervention were assessed with Kaplan-Meier survival analyses.RESULTS: Of 85 patients, 31 (36%) underwent PV repair. By multivariable analysis, longer PV total leaflet length (cm/m2) (beta=3.00, standard error [SE]=0.82, P<.001), larger PV z score (beta=1.34, SE=0.39, P=.001), and larger iPLA (beta=8.13, SE=2.62, P=.002) were associated with repair. iPLA of 0.90 or greater was 91% sensitive and 83% specific for achieving PV repair. At a median of 4.1years follow-up, there was greater freedom from significant PR in the PV repair group (log rank P=.008).CONCLUSIONS: Patients with an iPLA >0.9, and those with an iPLA between 0.7 and 0.9 with a PV annulus z score >0 should be considered for a native PV repair. At midterm follow-up, patients with a PV repair were not more likely to developPR or to require reintervention when compared with patients undergoing PVR.

  View details for DOI 10.1016/j.jtcvs.2019.06.110

  View details for PubMedID 31585750

• Patient size parameters to guide use of the Impella device in pediatric patients. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Morray, B. H., Dimas, V. V., McElhinney, D. B., Puri, K., Qureshi, A. M. 2019

  Abstract

  OBJECTIVES: To define patient and ventricular size parameters to guide Impella device (Abiomed, Inc., Danvers, MA) placement for mechanical circulatory support (MCS) in small pediatric patients (10-30kg).BACKGROUND: There are few options for MCS in children, and there are no data on minimum patient size requirements for placement of the Impella 2.5 device.METHODS: This was a multicenter study of echocardiographic and magnetic resonance imaging (MRI) derived dimensions of the left ventricle (LV) length and ascending aorta used to define minimum size parameters that are necessary for the placement of the Impella catheter.RESULTS: Data were collected from 44 cardiac MRIs conducted in healthy pediatric patients and 39 echocardiograms performed in pediatric patients with cardiomyopathy prior to surgical ventricular assist device (VAD) placement. The Impella 2.5 catheter is 7.5 cm from the pigtail to the aortic annulus marker, thus requiring an LV apical length of 7.5 cm to allow the device to function in an unconstrained fashion. In the cohort of patients undergoing consideration for VAD placement, a minimum LV length of 7.5 cm corresponded to a height of 122cm, weight of 23kg, and body surface area (BSA) of 0.89m2 . In the MRI cohort, this corresponded to a height of 121cm, weight of 23.9 kg, and BSA of 0.89m2 .CONCLUSION: MCS with Impella devices is feasible in pediatric patients. This study defines anthropomorphic and anatomic measurements to guide providers in patient selection for MCS using the Impella devices.

  View details for DOI 10.1002/ccd.28456

  View details for PubMedID 31424167

• A Real-Time Early Warning System for Monitoring Inpatient Mortality Risk: Prospective Study Using Electronic Medical Record Data. Journal of medical Internet research Ye, C., Wang, O., Liu, M., Zheng, L., Xia, M., Hao, S., Jin, B., Jin, H., Zhu, C., Huang, C. J., Gao, P., Ellrodt, G., Brennan, D., Stearns, F., Sylvester, K. G., Widen, E., McElhinney, D. B., Ling, X. 2019; 21 (7): e13719

  Abstract

  BACKGROUND: The rapid deterioration observed in the condition of some hospitalized patients can be attributed to either disease progression or imperfect triage and level of care assignment after their admission. An early warning system (EWS) to identify patients at high risk of subsequent intrahospital death can be an effective tool for ensuring patient safety and quality of care and reducing avoidable harm and costs.OBJECTIVE: The aim of this study was to prospectively validate a real-time EWS designed to predict patients at high risk of inpatient mortality during their hospital episodes.METHODS: Data were collected from the system-wide electronic medical record (EMR) of two acute Berkshire Health System hospitals, comprising 54,246 inpatient admissions from January 1, 2015, to September 30, 2017, of which 2.30% (1248/54,246) resulted in intrahospital deaths. Multiple machine learning methods (linear and nonlinear) were explored and compared. The tree-based random forest method was selected to develop the predictive application for the intrahospital mortality assessment. After constructing the model, we prospectively validated the algorithms as a real-time inpatient EWS for mortality.RESULTS: The EWS algorithm scored patients' daily and long-term risk of inpatient mortality probability after admission and stratified them into distinct risk groups. In the prospective validation, the EWS prospectively attained a c-statistic of 0.884, where 99 encounters were captured in the highest risk group, 69% (68/99) of whom died during the episodes. It accurately predicted the possibility of death for the top 13.3% (34/255) of the patients at least 40.8 hours before death. Important clinical utilization features, together with coded diagnoses, vital signs, and laboratory test results were recognized as impactful predictors in the final EWS.CONCLUSIONS: In this study, we prospectively demonstrated the capability of the newly-designed EWS to monitor and alert clinicians about patients at high risk of in-hospital death in real time, thereby providing opportunities for timely interventions. This real-time EWS is able to assist clinical decision making and enable more actionable and effective individualized care for patients' better health outcomes in target medical facilities.

  View details for DOI 10.2196/13719

  View details for PubMedID 31278734

• Prediction of the 1-Year Risk of Incident Lung Cancer: Prospective Study Using Electronic Health Records from the State of Maine JOURNAL OF MEDICAL INTERNET RESEARCH Wang, X., Zhang, Y., Hao, S., Zheng, L., Liao, J., Ye, C., Xia, M., Wang, O., Liu, M., Weng, C., Duong, S. Q., Jin, B., Alfreds, S. T., Stearns, F., Kanov, L., Sylvester, K. G., Widen, E., McElhinney, D. B., Ling, X. B. 2019; 21 (5)

  View details for DOI 10.2196/13260

  View details for Web of Science ID 000468102900001

• Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals CARDIOLOGY IN THE YOUNG Wise-Faberowski, L., Irvin, M., Sidell, D. R., Rajashekara, S., Asija, R., Chan, F. P., Hanley, F. L., McElhinney, D. B., Pulm Artery Reconstruction Program 2019; 29 (5): 610–14

  View details for DOI 10.1017/S1047951119000301

  View details for Web of Science ID 000472634800009

• Tetralogy of Fallot: Everything you wanted to know but were afraid to ask PEDIATRIC ANESTHESIA Wise-Faberowski, L., Asija, R., McElhinney, D. B. 2019; 29 (5): 475–82

  View details for DOI 10.1111/pan.13569

  View details for Web of Science ID 000468217100012

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring CONGENITAL HEART DISEASE Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019; 14 (3): 410–18

  View details for DOI 10.1111/chd.12736

  View details for Web of Science ID 000471070900013

• Echocardiographic Evaluation of Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Replacement JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Tompkins, R., Kelle, A. M., Cabalka, A. K., Lui, G. K., Aboulhosn, J., Dvir, D., McElhinney, D. B., VIVID Registry 2019; 32 (5): 616–23

  View details for DOI 10.1016/j.echo.2018.12.008

  View details for Web of Science ID 000466616400006

• Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY Wise-Faberowski, L., Irvin, M., Lennig, M., Long, J., Nadel, H. R., Bauser-Heaton, H., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 10 (3): 313–20

  View details for DOI 10.1177/2150135119836735

  View details for Web of Science ID 000470668700010

• Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. World journal for pediatric & congenital heart surgery Wise-Faberowski, L., Irvin, M., Lennig, M., Long, J., Nadel, H. R., Bauser-Heaton, H., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 10 (3): 313–20

  Abstract

  BACKGROUND: Pulmonary vascular supply in tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) is highly variable. Our approach to surgical management of this condition emphasizes early repair including unifocalization and reconstruction of the pulmonary circulation, incorporating all lung segments and addressing stenoses both proximal to and within the lung, in addition to ventricular septal defect closure. At our institution, we have over 15 years of experience using lung perfusion scintigraphy (LPS) to assess the distribution of pulmonary blood flow after complete unifocalization and repair.METHODS: We reviewed clinical and quantitative LPS data in 310 patients who underwent complete unifocalization and repair of TOF/MAPCAs from 2003 to 2018 at our institution. Postrepair relative lung perfusion distributions were determined from LPS initially obtained at our institution within 60 days after repair and thereafter.RESULTS: Total lung perfusion to the right and left lungs was 58.0% ± 14.2% and 42.0% ± 14.2%, respectively. Perfusion was balanced in 75% of patients and unbalanced in 25%, including 11% in whom it was extremely unbalanced. On multivariable analysis, older age at repair, surgery other than a single-stage complete unifocalization, and native anatomy consisting of unilateral pulmonary blood supply through a ductus arteriosus were associated with unbalanced perfusion.CONCLUSION: We present our experience using LPS as an outcome measure after surgical repair of TOF/MAPCAs. Balanced lung perfusion was present in the majority of patients who had complete repair of TOF/MAPCAs performed at our center.

  View details for PubMedID 31084304

• Substantial Cardiovascular Morbidity in Adults With Lower-Complexity Congenital Heart Disease CIRCULATION Saha, P., Potiny, P., Rigdon, J., Morello, M., Tcheandjieu, C., Romfh, A., Fernandes, S. M., McElhinney, D. B., Bernstein, D., Lui, G. K., Shaw, G. M., Ingelsson, E., Priest, J. R. 2019; 139 (16): 1889–99

  View details for DOI 10.1161/CIRCULATIONAHA.118.037064

  View details for Web of Science ID 000469321500006

• Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair. Congenital heart disease Annavajjhala, V., Punn, R., Tacy, T. A., Hanley, F. L., McElhinney, D. B. 2019

  Abstract

  BACKGROUND: Little is known about the early time course of biventricular function and mechanics after tetralogy of Fallot (TOF) repair. We sought to evaluate and describe the evolution of the right ventricle (RV) after TOF repair in young infants and children using conventional echocardiographic parameters and global longitudinal strain (GLS).METHODS: A retrospective review was performed of all patients with TOF and pulmonary stenosis who underwent repair from January 2002 to September 2015 and had at least 3 serial postsurgical echocardiograms spanning from infancy to early childhood (<8 years). Student's t test was performed to compare patients who underwent valve sparing (VS) versus transannular patch (TAP) repair. ANOVA was used to track measures of ventricular systolic function over time.RESULTS: We analyzed 151 echocardiograms performed on 42 patients. Pulmonary regurgitation (PR, moderate or severe) and the RV to left ventricular (LV) basal dimension ratio were higher in TAP patients (P<.04 at all-time points). Along with a significant increase in RV basal diameter Z-score in the TAP group (P<.001), there was an improvement in RV and LV GLS over time in both groups (P<.001). The LV GLS at last follow-up was lower in patients who underwent reoperation than those who did not (P=.050). LV GLS at the last follow-up echocardiogram was lower in patients with significant PR than those without (P<.001).CONCLUSIONS: Ventricular function appeared improve over time from the initial postoperative period in TOF patients. TAP repair was associated with a progressively higher RV/LV ratio in young children. GLS and RV/LV basal diameter ratio may be useful when following young children after TOF repair. Further research is necessary to understand the trajectory of ventricular functional and volumetric changes in young children in order to provide the most effective lifetime management of patients with TOF.

  View details for PubMedID 30989806

• Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals. The Annals of thoracic surgery Bauser-Heaton, H., Ma, M., McElhinney, D. B., Goodyer, W. R., Zhang, Y., Chan, F. P., Asija, R., Shek, J., Wise-Faberowski, L., Hanley, F. L. 2019

  Abstract

  BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR, and mid-term outcomes have not been assessed.METHODS: Clinical data were reviewed. PA diameters were measured off-line from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions.RESULTS: From 11/01-3/18, 352 patients with TOF/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median 8 months). There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (0.22-0.74).CONCLUSIONS: Most patients with TOF/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.

  View details for PubMedID 30980823

• Cardiac Resynchronization Therapy in Congenital Heart Disease and Pediatric Patients with Heart Failure is Associated with Improved Survival Chubb, H., Almond, C. S., Rosenthal, D. N., Trela, A. V., Hanisch, D., Motonaga, K. S., Ceresnak, S. R., McElhinney, D. B., Dubin, A. M. ELSEVIER SCIENCE INC. 2019: S201–S202

  View details for DOI 10.1016/j.healun.2019.01.488

  View details for Web of Science ID 000461365101175

• Repair of Untreated Older Patients With Tetralogy of Fallot With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Vaikunth, S. S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 107 (4): 1218–25

  View details for DOI 10.1016/j.athoracsur.2018.11.016

  View details for Web of Science ID 000462308000055

• Substantial Cardiovascular Morbidity in Adults with Lower-Complexity Congenital Heart Disease. Circulation Saha, P., Potiny, P., Rigdon, J., Morello, M., Tcheandjieu, C., Romfh, A., Fernandes, S. M., McElhinney, D. B., Bernstein, D., Lui, G. K., Shaw, G. M., Ingelsson, E., Priest, J. R. 2019

  Abstract

  BACKGROUND: Although lower-complexity cardiac malformations constitute the majority of adult congenital heart disease (ACHD), the long-term risks of adverse cardiovascular events and relationship with conventional risk factors in this population are poorly understood. We aimed to quantify the risk of adverse cardiovascular events associated with lower-complexity ACHD that is unmeasured by conventional risk factors.METHODS: A multi-tiered classification algorithm was used to select individuals with lower-complexity ACHD and individuals without ACHD for comparison amongst >500,000 British adults in the UK Biobank (UKB). ACHD diagnoses were sub-classified as "isolated aortic valve (AoV)" and "non-complex" defects. Time-to-event analyses were conducted for primary endpoints of fatal or non-fatal acute coronary syndrome (ACS), ischemic stroke, heart failure (HF), and atrial fibrillation, and a secondary combined endpoint for major adverse cardiovascular event (MACE). Maximum follow-up time for the study period was 22 years using retrospectively and prospectively collected data from the UKB.RESULTS: We identified 2,006 individuals with lower-complexity ACHD and 497,983 unexposed individuals in the UKB (median [IQR] age at enrollment 58 [51,63]). Of the ACHD-exposed group, 59% were male; 51% were current or former smokers; 30% were obese; 69%, 41%, and 7% were diagnosed or treated for hypertension, hyperlipidemia, and diabetes respectively. After adjustment for 12 measured cardiovascular risk factors, ACHD remained strongly associated with the primary endpoints, with hazard ratios (HR) ranging from 2.0 (95% confidence interval [CI] 1.5-2.8, p<0.001) for ACS to 13.0 (95% CI 9.4-18.1, p<0.001) for HF. ACHD-exposed individuals with ≤2 cardiovascular risk factors had a 29% age-adjusted incidence rate of MACE in contrast to 13% in non-ACHD individuals with ≥5 risk factors.CONCLUSIONS: Individuals with lower-complexity ACHD had higher burden of adverse cardiovascular events relative to the general population that was unaccounted for by conventional cardiovascular risk factors. These findings highlight the need for closer surveillance of patients with mild to moderate ACHD and further investigation into management and mechanisms of cardiovascular risk unique to this growing population of high-risk adults.

  View details for PubMedID 30813762

• Echocardiographic Evaluation of Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Replacement. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Tompkins, R., Kelle, A. M., Cabalka, A. K., Lui, G. K., Aboulhosn, J., Dvir, D., McElhinney, D. B., VIVID Registry 2019

  Abstract

  BACKGROUND: Transcatheter tricuspid valve-in-valve replacement (TVIV) is an emerging therapy for dysfunctional surgical valves in patients with congenital and acquired TV disease. The present study was performed to establish baseline quantitative data for echocardiographic and invasive parameters obtained pre- and immediately post-TVIV.METHODS: Patients were drawn from the VIVID Registry. This study included two cohorts. The registry cohort included all patients entered in the VIVID registry through February 2017 who had both echocardiographic and invasively measured gradients across the TV. The focused cohort comprised a subset of patients from a single institution who had both pre- and post-TVIV echocardiogram images reviewed offline by a single investigator. The echocardiographic variables measured were based on published guidelines from the American Society of Echocardiography.RESULTS: Assessment of paired pre- and/or postimplant echocardiographic and invasive pressure measurements (n=199) showed reasonable correlation between mean TV gradient measured invasively with cardiac catheterization and noninvasively both pre- and post-TVIV (R=0.72, P<.001), although there was a bias toward the echocardiographic gradient being higher than the invasively measured gradient and sizable discrepancies were reported in several patients. In the focused cohort (n=42), the mean TV inflow gradient was 9.3±5.0mm Hg pre- and 5.6±2.3mm Hg post-TVIV (P<.001). The TV pressure halftime and TV:left ventricular outflow tract Doppler velocity index were 215±94 msec and 3.4±1.2, respectively, at baseline, and 170±44 msec and 2.4±0.6 post-TVIV. Both the Doppler velocity index and the TVE velocity correlated with the mean TV inflow gradient.CONCLUSIONS: This study provides benchmark data for the echocardiographic assessment of valve function after TVIV. In this population, the significance of an inflow gradient after TVIV should be interpreted in the clinical context. The appropriate threshold for defining dysfunction may differ from the levels proposed for assessment of native or newly placed surgical valves.

  View details for PubMedID 30773424

• Birth Location of Infants with Critical Congenital Heart Disease in California PEDIATRIC CARDIOLOGY Purkey, N. J., Axelrod, D. M., McElhinney, D. B., Rigdon, J., Qin, F., Desai, M., Shin, A. Y., Chock, V. Y., Lee, H. C. 2019; 40 (2): 310–18

  View details for DOI 10.1007/s00246-018-2019-0

  View details for Web of Science ID 000460553100007

• Long-term surveillance biopsy: Is it necessary after pediatric heart transplant? PEDIATRIC TRANSPLANTATION Peng, D. M., Ding, V. Y., Hollander, S. A., Khalapyan, T., Dykes, J. C., Rosenthal, D. N., Almond, C. S., Sakarovitch, C., Desai, M., McElhinney, D. B. 2019; 23 (1)

  View details for DOI 10.1111/petr.13330

  View details for Web of Science ID 000457579500023

• Mid-Term Valve-Related Outcomes After Transcatheter Tricuspid Valve-in-Valve or Valve-in-Ring Replacement JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Aboulhosn, J. A., Dvir, D., Whisenant, B., Zhang, Y., Eicken, A., Ribichini, F., Tzifa, A., Hainstock, M. R., Martin, M. H., Kornowski, R., Schubert, S., Latib, A., Thomson, J. R., Torres, A. J., Meadows, J., Delaney, J. W., Guerrero, M. E., Salizzoni, S., El-Said, H., Finkelstein, A., George, I., Gewillig, M., Alvarez-Fuente, M., Lamers, L., Cheema, A. N., Kreutzer, J. N., Rudolph, T., Hildick-Smith, D., Cabalka, A. K., VIVID Registry 2019; 73 (2): 148–57

  Abstract

  Transcatheter aortic and pulmonary valves have been used to treat stenosis or regurgitation after prior surgical tricuspid valve (TV) replacement or repair. Little is known about intermediate-term valve-related outcomes after transcatheter tricuspid valve replacement (TTVR), including valve function, thrombus, and endocarditis.The authors sought to evaluate mid-term outcomes in a large cohort of patients who underwent TTVR after surgical TV repair or replacement, with a focus on valve-related outcomes.Patients who underwent TTVR after prior surgical TV replacement or repair were collected through an international registry. Time-related outcomes were modeled and risk factors assessed.Data were collected for 306 patients who underwent TTVR from 2008 through 2017 at 80 centers; 52 patients (17%) had a prior history of endocarditis. Patients were followed for a median of 15.9 months after implantation (0.1 to 90 months), with 64% of patients estimated to be alive without TV reintervention or a valve-related event at 3 years. The cumulative 3-year incidence of death, reintervention, and valve-related adverse outcomes (endocarditis, thrombosis, or significant dysfunction) were 17%, 12%, and 8%, respectively. Endocarditis was diagnosed in 8 patients 2 to 29 months after TTVR, for an annualized incidence rate of 1.5% per patient-year (95% confidence interval: 0.45% to 2.5%). An additional 8 patients were diagnosed with clinically relevant valve thrombosis, 3 in the short term, 2 within 2 months, and 3 beyond 6 months. Only 2 of these 8 patients received anticoagulant therapy before thrombus detection (p = 0.13 vs. patients without thrombus). Prior endocarditis was not a risk factor for reintervention, endocarditis, or valve thrombosis, and there was no difference in valve-related outcomes according to TTVR valve type.TV dysfunction, endocarditis, and leaflet thrombosis were uncommon after TTVR. Patients with prior endocarditis were not at higher risk for endocarditis or other adverse outcomes after TTVR, and endocarditis occurred with similar frequency in different valve types. Though rare, leaflet thrombosis is an important adverse outcome, and further study is necessary to determine the appropriate level of prophylactic therapy after TTVR.

  View details for PubMedID 30654886

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring. Congenital heart disease Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019

  Abstract

  BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

  View details for PubMedID 30604934

• A proteomic clock for malignant gliomas: The role of the environment in tumorigenesis at the presymptomatic stage. PloS one Zheng, L. n., Zhang, Y. n., Hao, S. n., Chen, L. n., Sun, Z. n., Yan, C. n., Whitin, J. C., Jang, T. n., Merchant, M. n., McElhinney, D. B., Sylvester, K. G., Cohen, H. J., Recht, L. n., Yao, X. n., Ling, X. B. 2019; 14 (10): e0223558

  Abstract

  Malignant gliomas remain incurable with a poor prognosis despite of aggressive treatment. We have been studying the development of brain tumors in a glioma rat model, where rats develop brain tumors after prenatal exposure to ethylnitrosourea (ENU), and there is a sizable interval between when the first pathological changes are noted and tumors become detectable with MRI. Our aim to define a molecular timeline through proteomic profiling of the cerebrospinal fluid (CSF) such that brain tumor commitment can be revealed earlier than at the presymptomatic stage. A comparative proteomic approach was applied to profile CSF collected serially either before, at and after the time MRI becomes positive. Elastic net (EN) based models were developed to infer the timeline of normal or tumor development respectively, mirroring a chronology of precisely timed, "clocked", adaptations. These CSF changes were later quantified by longitudinal entropy analyses of the EN predictive metric. False discovery rates (FDR) were computed to control the expected proportion of the EN models that are due to multiple hypothesis testing. Our ENU rat brain tumor dating EN model indicated that protein content in CSF is programmed even before tumor MRI detection. The findings of the precisely timed CSF tumor microenvironment changes at presymptomatic stages, deviation from the normal development timeline, may provide the groundwork for the understanding of adaptation of the brain environment in tumorigenesis to devise effective brain tumor management strategies.

  View details for DOI 10.1371/journal.pone.0223558

  View details for PubMedID 31600288

• Association between patient age at implant and outcomes after transcatheter pulmonary valve replacement in the multicenter Melody valve trials. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Armstrong, A. K., Berger, F. n., Jones, T. K., Moore, J. W., Benson, L. N., Cheatham, J. P., Turner, D. R., Rhodes, J. F., Vincent, J. A., Zellers, T. n., Lung, T. H., Eicken, A. n., McElhinney, D. B. 2019

  Abstract

  To investigate whether age and valve size at implant contribute to outcomes after Melody transcatheter pulmonary valve replacement (TPVR).Patient age and valve size at implant contribute to longevity of surgical pulmonary valves.All patients discharged with a Melody valve in the pulmonary position, as part of three prospective Melody valve multicenter studies, comprised the study cohort. Acute and time-related outcomes were analyzed according to age: children (≤12 years), adolescents (13-18 years), young adults (19-29 years), and older adults (≥30 years).Successful Melody valve implantation occurred in 49 children, 107 adolescents, 96 young adults, and 57 older adults. Pediatric patients (≤18 years) were more likely to have TPVR for conduit stenosis than adults (62% vs. 44%); children had the smallest conduits. After TPVR, pediatric and adult patients had similar decreases in right ventricular (RV) size by MRI, but adults had improved percentage predicted peak VO2 (58% preimplant to 64% postimplant, p = .02) and FEV1 (69% pre to 71% post, p = .005). Younger age was associated with shorter freedom from RVOT dysfunction, reintervention, and explant. Children had the shortest freedom from endocarditis (p = .041), but all other groups had 5-year freedom from endocarditis of ≥90%.Younger age was associated with shorter time to RVOT dysfunction, reintervention, and explant after Melody TPVR. Patients ≥13 years of age were at low risk for endocarditis and explant to 5 years. A better understanding of time-related outcomes by age will aid in the comparison of therapeutic options for TPVR candidates.https://clinicaltrials.gov/ct2/show/NCT00740870 (NCT00740870), https://clinicaltrials.gov/ct2/show/NCT01186692 (NCT01186692), and https://clinicaltrials.gov/ct2/show/NCT00688571 (NCT00688571).

  View details for DOI 10.1002/ccd.28454

  View details for PubMedID 31419019

• Pulmonary hemorrhage in children with Alagille syndrome undergoing cardiac catheterization. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Feinstein, J. A., Yarlagadda, V. V., Lin, A. n., Wise-Faberowski, L. n., McElhinney, D. B. 2019

  Abstract

  To evaluate the incidence, severity, and outcomes of pulmonary hemorrhage in children with Alagille syndrome (AGS) undergoing cardiac catheterization, and to find variables associated with hemorrhage in this population.Children with AGS have a high incidence of bleeding complications during invasive procedures. It has been our impression that catheterization-associated pulmonary hemorrhage is more common in children with AGS, but there are no published data on this topic.This was a retrospective single institution study of children with AGS undergoing catheterization from 2010 to 2018. Pulmonary hemorrhage was defined as angiographic or fluoroscopic evidence of extravasated blood in the lung parenchyma, or blood suctioned from the endotracheal tube with documentation of pulmonary hemorrhage by the anesthesiologist or intensivist. Univariate comparisons were made between catheterizations that did and did not have pulmonary hemorrhage.Thirty children with AGS underwent 87 catheterizations, 32 (37%) with interventions on the branch pulmonary arteries (PA). There were 26 (30%) procedures with hemorrhage, the majority (65%) of which were self-limited or required less than 24 hr of mechanical ventilation. Moderate and severe hemorrhage occurred only in children with tetralogy of Fallot (TOF; 5 of 14, 36%). A higher right ventricle to aorta systolic pressure ratio (1.0 [0.85-1.1] vs. 0.88 [0.59-1.0], p = .029) and interventions on the branch PAs (14 of 26, 54% vs. 18 of 61, 30%, p = .032) were associated with hemorrhage.Pulmonary hemorrhage was common in children with AGS undergoing both intervention and diagnostic cardiac catheterization, and was associated with TOF, higher RV to aorta pressure ratio, and interventions on the branch PAs.

  View details for DOI 10.1002/ccd.28508

  View details for PubMedID 31584246

• Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. Cardiology in the young Wise-Faberowski, L. n., Irvin, M. n., Sidell, D. R., Rajashekara, S. n., Asija, R. n., Chan, F. P., Hanley, F. L., McElhinney, D. B. 2019: 1–5

  Abstract

  Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy.The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined.Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3.This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

  View details for PubMedID 31044684

• Prediction of the 1-Year Risk of Incident Lung Cancer: Prospective Study Using Electronic Health Records from the State of Maine. Journal of medical Internet research Wang, X. n., Zhang, Y. n., Hao, S. n., Zheng, L. n., Liao, J. n., Ye, C. n., Xia, M. n., Wang, O. n., Liu, M. n., Weng, C. H., Duong, S. Q., Jin, B. n., Alfreds, S. T., Stearns, F. n., Kanov, L. n., Sylvester, K. G., Widen, E. n., McElhinney, D. B., Ling, X. B. 2019; 21 (5): e13260

  Abstract

  Lung cancer is the leading cause of cancer death worldwide. Early detection of individuals at risk of lung cancer is critical to reduce the mortality rate.The aim of this study was to develop and validate a prospective risk prediction model to identify patients at risk of new incident lung cancer within the next 1 year in the general population.Data from individual patient electronic health records (EHRs) were extracted from the Maine Health Information Exchange network. The study population consisted of patients with at least one EHR between April 1, 2016, and March 31, 2018, who had no history of lung cancer. A retrospective cohort (N=873,598) and a prospective cohort (N=836,659) were formed for model construction and validation. An Extreme Gradient Boosting (XGBoost) algorithm was adopted to build the model. It assigned a score to each individual to quantify the probability of a new incident lung cancer diagnosis from October 1, 2016, to September 31, 2017. The model was trained with the clinical profile in the retrospective cohort from the preceding 6 months and validated with the prospective cohort to predict the risk of incident lung cancer from April 1, 2017, to March 31, 2018.The model had an area under the curve (AUC) of 0.881 (95% CI 0.873-0.889) in the prospective cohort. Two thresholds of 0.0045 and 0.01 were applied to the predictive scores to stratify the population into low-, medium-, and high-risk categories. The incidence of lung cancer in the high-risk category (579/53,922, 1.07%) was 7.7 times higher than that in the overall cohort (1167/836,659, 0.14%). Age, a history of pulmonary diseases and other chronic diseases, medications for mental disorders, and social disparities were found to be associated with new incident lung cancer.We retrospectively developed and prospectively validated an accurate risk prediction model of new incident lung cancer occurring in the next 1 year. Through statistical learning from the statewide EHR data in the preceding 6 months, our model was able to identify statewide high-risk patients, which will benefit the population health through establishment of preventive interventions or more intensive surveillance.

  View details for PubMedID 31099339

• Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot with MAPCAs. The Annals of thoracic surgery Bauser-Heaton, H. n., Ma, M. n., Wise-Faberowski, L. n., Asija, R. n., Shek, J. n., Zhang, Y. n., Peng, L. F., Sidell, D. R., Hanley, F. L., McElhinney, D. B. 2019

  Abstract

  Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair.Patients with TOF/MAPCAs who underwent unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size.From 11/01-12/17, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n=16) and/or at our center (n=7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Survival was 74±6% 5 years after unifocalization. At follow-up, the median PA:aortic systolic pressure ratio was 0.36 and was >0.50 in 2 patients.In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.

  View details for DOI 10.1016/j.athoracsur.2019.01.030

  View details for PubMedID 30772338

• Validation of a novel automated signal analysis tool for ablation of Wolff-Parkinson-White Syndrome. PloS one Ceresnak, S. R., Pass, R. H., Dubin, A. M., Yang, L., Motonaga, K. S., Hedlin, H., Avasarala, K., Trela, A., McElhinney, D. B., Janson, C., Nappo, L., Ling, X. B., Gates, G. J. 2019; 14 (6): e0217282

  Abstract

  BACKGROUND: In previous pilot work we demonstrated that a novel automated signal analysis tool could accurately identify successful ablation sites during Wolff-Parkinson-White (WPW) ablation at a single center.OBJECTIVE: We sought to validate and refine this signal analysis tool in a larger multi-center cohort of children with WPW.METHODS: A retrospective review was performed of signal data from children with WPW who underwent ablation at two pediatric arrhythmia centers from 2008-2015. All patients with WPW ≤ 21 years who underwent invasive electrophysiology study and ablation with ablation signals available for review were included. Signals were excluded if temperature or power delivery was inadequate or lesion time was < 5 seconds. Ablation lesions were reviewed for each patient. Signals were classified as successful if there was loss of antegrade and retrograde accessory pathway (AP) conduction or unsuccessful if ablation did not eliminate AP conduction. Custom signal analysis software analyzed intracardiac electrograms for amplitudes, high and low frequency components, integrated area, and signal timing components to create a signal score. We validated the previously published signal score threshold 3.1 in this larger, more diverse cohort and explored additional scoring options. Logistic regression with lasso regularization using Youden's index criterion and a cost-benefit criterion to identify thresholds was considered as a refinement to this score.RESULTS: 347 signals (141 successful, 206 unsuccessful) in 144 pts were analyzed [mean age 13.2 ± 3.9 years, 96 (67%) male, 66 (45%) left sided APs]. The software correctly identified the signals as successful or unsuccessful in 276/347 (80%) at a threshold of 3.1. The performance of other thresholds did not significantly improve the predictive ability. A signal score threshold of 3.1 provided the following diagnostic accuracy for distinguishing a successful from unsuccessful signal: sensitivity 83%, specificity 77%, PPV 71%, NPV 87%.CONCLUSIONS: An automated signal analysis software tool reliably distinguished successful versus unsuccessful ablation electrograms in children with WPW when validated in a large, diverse cohort. Refining the tools using an alternative threshold and statistical method did not improve the original signal score at a threshold of 3.1. This software was effective across two centers and multiple operators and may be an effective tool for ablation of WPW.

  View details for DOI 10.1371/journal.pone.0217282

  View details for PubMedID 31242221

• Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery CARDIOLOGY IN THE YOUNG Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2019; 29 (1): 19–22

  View details for DOI 10.1017/S1047951118001427

  View details for Web of Science ID 000454076300004

• Tetralogy of Fallot: Everything You Wanted to Know butWere Afraid to Ask. Paediatric anaesthesia Wise-Faberowski, L., Asija, R., McElhinney, D. B. 2018

  Abstract

  Tetralogy of Fallot (TOF) is the most common form of congenital cyanotic heart disease, occurring in approximately 4 to 5 per 10,0000 live births, and represents 7-10% of all congenital heart defects (1,2). Although TOF is often thought of in terms of the tetrad of anomalies-pulmonary stenosis, ventricular septal defect (VSD), aorta overriding the ventricular septum, and right ventricular hypertrophy-it has been proposed that all of these features are the result of anterior malalignment of the infundibular septum with the muscular septum (3,4). There are several variants of TOF as well, which manifest other abnormal features and are discussed in the following review. This article is protected by copyright. All rights reserved.

  View details for PubMedID 30592107

• Repair of Untreated Older Patients with Tetralogy of Fallot with Major Aortopulmonary Collaterals. The Annals of thoracic surgery Vaikunth, S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2018

  Abstract

  BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals.METHODS: Any patient with this lesion not treated prior to 2 years of age referred to our center from 2002-17 met inclusion criteria.RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n=11) were >9 years old, had polycythemia, and/or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n=31) of patients, 82% (n=27) in one stage and 12% (n=4) after unifocalization to a central shunt. The median right ventricular to aortic pressure ratio was 0.31 after surgery and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement.CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable to infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to healthcare providers beyond infancy should be considered candidates and evaluated for complete repair.

  View details for PubMedID 30550802

• Transcatheter Aortic Valve Replacement With a Repositionable Self-Expanding Prosthesis The PORTICO-I Trial 1-Year Outcomes JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Sondergaard, L., Rodes-Cabau, J., Linke, A., Fichtlscherer, S., Schaefer, U., Kuck, K., Kempfert, J., Arzamendi, D., Bedogni, F., Asch, F. M., Worthley, S., Maisano, F. 2018; 72 (23): 2859–67

  View details for DOI 10.1016/j.jacc.2018.09.014

  View details for Web of Science ID 000452000900007

• Endocarditis After Transcatheter Pulmonary Valve Replacement. Journal of the American College of Cardiology McElhinney, D. B., Sondergaard, L., Armstrong, A. K., Bergersen, L., Padera, R. F., Balzer, D. T., Lung, T., Berger, F., Zahn, E. M., Gray, R. G., Hellenbrand, W. E., Kreutzer, J., Eicken, A., Jones, T. K., Ewert, P. 2018; 72 (22): 2717–28

  Abstract

  BACKGROUND: Endocarditis has emerged as one of the most concerning adverse outcomes in patients with congenital anomalies involving the right ventricular outflow tract (RVOT) and prosthetic valves.OBJECTIVES: The aim of this study was to evaluate rates and potential risk factors for endocarditis after transcatheter pulmonary valve replacement in the prospective Melody valve trials.METHODS: All patients in whom a transcatheter pulmonary valve (TPV) was implanted in the RVOT as part of 3 prospective multicenter studies comprised the analytic cohort. The diagnosis of endocarditis and involvement of the TPV were determined by the implanting investigator.RESULTS: A total of 309 patients underwent transcatheter pulmonary valve replacement (TPVR) and were discharged with a valve in place. The median follow-up duration was 5.1 years, and total observation until study exit was1,660.3patient-years. Endocarditis was diagnosed in 46 patients (median 3.1 years after TPVR), and a total of 35patients were reported to have TPV-related endocarditis (34 at the initial diagnosis, 1 with a second episode). Theannualized incidence rate of endocarditis was 3.1% per patient-year and of TPV-related endocarditis was 2.4% per patient-year. At 5 years post-TPVR, freedom from a diagnosis of endocarditis was 89% and freedom from TPV-related endocarditis was 92%. By multivariable analysis, age≤12 years at implant (hazard ratio: 2.3; 95% confidence interval: 1.2to 4.4; p=0.011) and immediate post-implant peak gradient≥15mmHg (2.7;95% confidence interval: 1.4 to 4.9; p=0.002) were associated with development of endocarditis and with development of TPV-related endocarditis (age≤12 years: 2.8;95% confidence interval: 1.3to 5.7; p=0.006; gradient≥15mmHg: 2.6; 95% confidence interval: 1.3 to 5.2; p=0.008).CONCLUSIONS: Endocarditis is an important adverse outcome following TVPR in children and adults with post-operativecongenital heart disease involving the RVOT. Ongoing efforts to understand, prevent, and optimize management of thiscomplication are paramount in making the best use of TPV therapy. (Melody Transcatheter Pulmonary Valve [TPV] Study: Post Approval Study of the Original Investigational Device Exemption [IDE] Cohort; NCT00740870; MelodyTranscatheter Pulmonary Valve Post-Approval Study; NCT01186692; and Melody Transcatheter PulmonaryValve[TPV] Post-Market Surveillance Study; NCT00688571).

  View details for DOI 10.1016/j.jacc.2018.09.039

  View details for PubMedID 30497557

• Long-term surveillance biopsy: Is it necessary after pediatric heart transplant? Pediatric transplantation Peng, D. M., Ding, V. Y., Hollander, S. A., Khalapyan, T., Dykes, J. C., Rosenthal, D. N., Almond, C. S., Sakarovitch, C., Desai, M., McElhinney, D. B. 2018: e13330

  Abstract

  Due to limited and conflicting data in pediatric patients, long-term routine surveillance endomyocardial biopsy (RSB) in pediatric heart transplant (HT) remains controversial. We sought to characterize the rate of positive RSB and determine factors associated with RSB-detected rejection. Records of patients transplanted at a single institution from 1995 to 2015 with >2year of post-HT biopsy data were reviewed for RSB-detected rejections occurring >2year post-HT. We illustrated the trajectory of significant rejections (ISHLT Grade ≥3A/2R) among total RSB performed over time and used multivariable logistic regression to model the association between time and risk of rejection. We estimated Kaplan-Meier freedom from rejection rates by patient characteristics and used the log-rank test to assess differences in rejection probabilities. We identified the best-fitting Cox proportional hazards regression model. In 140 patients, 86% did not have any episodes of significant RSB-detected rejection >2year post-HT. The overall empirical rate of RSB-detected rejection >2year post-HT was 2.9/100 patient-years. The percentage of rejection among 815 RSB was 2.6% and remained stable over time. Years since transplant remained unassociated with rejection risk after adjusting for patient characteristics (OR=0.98; 95% CI 0.78-1.23; P=0.86). Older age at HT was the only factor that remained significantly associated with risk of RSB-detected rejection under multivariable Cox analysis (P=0.008). Most pediatric patients did not have RSB-detected rejection beyond 2years post-HT, and the majority of those who did were older at time of HT. Indiscriminate long-term RSB in pediatric heart transplant should be reconsidered given the low rate of detected rejection.

  View details for PubMedID 30506612

• Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Rosner, A., Khalapyan, T., Pedrosa, J., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 35 (12): 2035–46

  View details for DOI 10.1111/echo.14169

  View details for Web of Science ID 000451879200016

• Transcatheter pulmonary valve replacement: evolving indications and application. Future cardiology Shahanavaz, S., McElhinney, D. B. 2018

  Abstract

  The introduction of transcatheter therapy for valvular heart disease has changed the spectrum of care of patients with a variety of cardiovascular conditions. Transcatheter valve placement has become established as a method of treating pathologic regurgitation or stenosis of the pulmonary valve, right ventricular outflow tract or a right ventricle to pulmonary artery conduit. In this review, we examine the pathophysiology of and indications for transcatheter pulmonary valve replacement along with procedural complications. Advancements in clinical application and valve technology will also be covered.

  View details for PubMedID 30499688

• Mortality prediction after transcatheter treatment of failed bioprosthetic aortic valves utilizing various international scoring systems: Insights from the Valve-in-Valve International Data (VIVID) CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Aziz, M., Simonato, M., Webb, J. G., Abdel-Wahab, M., McElhinney, D., Duncan, A., Tchetche, D., Barbanti, M., Petronio, A., Maisano, F., Ribeiro, V., Gaia, D., Rana, R., Kocka, V., Mathur, M., Wijeysundera, H., Hellig, F., Nissen, H., Bekeredjian, R., Rihal, C., Duffy, S. J., Dvir, D. 2018; 92 (6): 1163–70

  View details for DOI 10.1002/ccd.27714

  View details for Web of Science ID 000451188300032

• Birth Location of Infants with Critical Congenital Heart Disease in California. Pediatric cardiology Purkey, N. J., Axelrod, D. M., McElhinney, D. B., Rigdon, J., Qin, F., Desai, M., Shin, A. Y., Chock, V. Y., Lee, H. C. 2018

  Abstract

  The American Academy of Pediatrics classifies neonatal intensive care units (NICUs) from level I to IV based on the acuity of care each unit can provide. Birth in a higher level center is associated with lower morbidity and mortality in high-risk populations. Congenital heart disease accounts for 25-50% of infant mortality related to birth defects in the U.S., but recent data are lacking on where infants with critical congenital heart disease (CCHD) are born. We used a linked dataset from the Office of Statewide Health Planning and Development to access ICD-9 diagnosis codes for all infants born in California from 2008 to 2012. We compared infants with CCHD to the general population, identified where infants with CCHD were born based on NICU level of care, and predicted level IV birth among infants with CCHD using logistic regression techniques. From 2008 to 2012, 6325 infants with CCHD were born in California, with 23.7% of infants with CCHD born at a level IV NICU compared to 8.4% of the general population. Level IV birth for infants with CCHD was associated with lower gestational age, higher maternal age and education, the presence of other congenital anomalies, and the diagnosis of a single ventricle lesion. More infants with CCHD are born in a level IV NICU compared to the general population. Future studies are needed to determine if birth in a lower level of care center impacts outcomes for infants with CCHD.

  View details for PubMedID 30415381

• Secondary Repair of Incompetent Pulmonary Valves After Prior Surgery or Intervention: Patient Selection and Outcomes Adamson, G. T., McElhinney, D. B., Lui, G., Meadows, A. K., Hanley, F. L., Maskatia, S. A. LIPPINCOTT WILLIAMS & WILKINS. 2018

  View details for Web of Science ID 000528619403397

• Preliminary Evaluation of a Deep Learning Approach for Echocardiographic Screening for Rheumatic Heart Disease Edwards, L. A., Feng, F., Iqbal, M., Fu, Y., Sanyahumbi, A., Hao Shiying, McElhinney, D. B., Ling, X. B., Luo Jiajia LIPPINCOTT WILLIAMS & WILKINS. 2018

  View details for Web of Science ID 000528619407037

• Pre-Operative Right Ventricular Outflow Tract and Pulmonary Artery Geometry Predicts Pulmonary Valve Replacement Outcomes in Patients With Tetralogy of Fallot Toro Arana, V., Chan, F., Shiavone, N., McElhinney, D., Reddy, S., Marsden, A. LIPPINCOTT WILLIAMS & WILKINS. 2018

  View details for Web of Science ID 000528619407405

• Utility of screening echocardiogram after endomyocardial biopsy for identification of cardiac perforation or tricuspid valve injury. Pediatric transplantation Peng, D. M., Kipps, A. K., Palmon, M., Tacy, T. A., Peng, L. F., Hollander, S. A., McElhinney, D. B. 2018; 22 (7): e13275

  Abstract

  Per protocol, our institution obtains echocardiograms immediately after each EMB to rule out procedural complication. We sought to determine the incidence of echocardiogram-detected cardiac perforation and TV injury and to evaluate the utility of routine screening echocardiogram after each EMB in the current era. At a single center, 99% (1917/1942) EMB performed in 162 patients were immediately followed by an echocardiogram per protocol. There were five newly diagnosed pericardial effusions, and only one required pericardiocentesis. In the three echocardiograms demonstrating new flail TV, only one patient underwent surgical repair 2months later. This study demonstrates the very low incidence of significant hemopericardium and TV injury after EMB in pediatric heart transplant recipients and argues against the utility of post-EMB echocardiograms to screen solely for procedural complications.

  View details for PubMedID 30076684

• Transcatheter pulmonary valve replacement: evolving indications and application FUTURE CARDIOLOGY Shahanavaz, S., McElhinney, D. B. 2018; 14 (6): 511–24

  View details for DOI 10.2217/fca-2018-0065

  View details for Web of Science ID 000452378400005

• Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery - CORRIGENDUM. Cardiology in the young Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2018: 1

  View details for DOI 10.1017/S1047951118001804

  View details for PubMedID 30370896

• Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress. Echocardiography (Mount Kisco, N.Y.) Rosner, A., Khalapyan, T., Pedrosa, J., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018

  Abstract

  BACKGROUND: Patients with single ventricle physiology and Fontan circulation are at increased risk for late complications and reduced survival. The aim of the study was to investigate the correlation between ventricular geometry and systolic regional function in different underlying anatomic conditions in adolescent and adult Fontan-palliated patients.METHOD: In a retrospective cross-sectional study, we measured 2D strain, ventricular diameters, ventricular volumes, ejection fraction (EF), global and segmental wall stress, and sphericity index. The same analyses were performed in 99 age- and gender-matched healthy individuals.RESULTS: One hundred and one patients were included at a mean age of 21 (range 14-59) years. In comparison with healthy subjects, patients with Fontan circulation displayed larger ventricular volumes (153±78mL vs 116±38mL P<0.05), reduced EF (43%±15% vs 55%±8% P<0.05), reduced longitudinal (-13%±6% vs -21%±4% P<0.05) and circumferential strain values (-15%±7% vs -22%±4% P<0.05). Functionally single ventricles were more spherical (ratio of longitudinal to short-axis diameters 1.3±0.3 vs 1.7±0.2 P<0.05). Circumferential strain correlated well with global wall stress and the degree of sphericity (R2 =0.320), while segmental strain did not correlate with segmental wall stress. The percentage of segments with akinesia was relatively high (16±16% vs 0±0% P<0.05) indicating reduced segmental contractile function.CONCLUSION: Functionally single ventricles after Fontan palliation have significantly reduced systolic regional and global function with a high intersegmental inhomogeneity. The underlying pathological mechanisms might be multifactorial, including ventricular geometry, sphericity, and regional contractile properties. Future studies are needed to investigate the role of ventricular geometry for clinical performance and outcome.

  View details for PubMedID 30350438

• Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Koth, A. M., Kwiatkowski, D. M., Lim, T. R., Bauser-Heaton, H., Asija, R., McElhinney, D. B., Hanley, F. L., Krawczeski, C. D. 2018; 156 (3): 1181–87

  View details for DOI 10.1016/j.jtcvs.2018.04.088

  View details for Web of Science ID 000441556000071

• Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery. Cardiology in the young Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2018: 1–4

  Abstract

  BACKGROUND: A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.METHODS: We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.RESULTS: We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.CONCLUSION: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

  View details for PubMedID 30160647

• Safety and Feasibility of Melody Transcatheter Pulmonary Valve Replacement in the Native Right Ventricular Outflow Tract A Multicenter Pediatric Heart Network Scholar Study JACC-CARDIOVASCULAR INTERVENTIONS Martin, M., Meadows, J., McElhinney, D. B., Goldstein, B. H., Bergersen, L., Qureshi, A. M., Shahanavaz, S., Aboulhosn, J., Berman, D., Peng, L., Gillespie, M., Armstrong, A., Weng, C., Minich, L., Gray, R. G. 2018; 11 (16): 1642–50

  Abstract

  This study sought to determine the safety and feasibility of transcatheter pulmonary valve replacement (TPVR) using the Melody valve in native (nonconduit) right ventricular outflow tracts (nRVOT), and to identify factors associated with successful TPVR.The Melody valve is Food and Drug Administration-approved for TPVR within right ventricle-to-pulmonary artery conduits and bioprosthetic pulmonary valves. However, most patients needing pulmonary valve replacement have nRVOT and TPVR has been adapted for this indication.In this multicenter retrospective study of all patients presenting for nRVOT TPVR, we collected pre-procedural magnetic resonance imaging, echocardiography, and catheterization data, and evaluated procedural and early outcomes.Of 229 patients (age 21 ± 15 years from 11 centers), 132 (58%) had successful TPVR. In the remaining 97, TPVR was not performed, most often because of prohibitively large nRVOT (n = 67) or compression of the aortic root or coronary arteries (n = 18). There were no deaths and 5 (4%) serious complications, including pre-stent embolization requiring surgery in 4 patients, and arrhythmia in 1. Higher pre-catheterization echocardiographic RVOT gradient was associated with TPVR success (p = 0.001) and larger center volume approached significance (p = 0.08). Magnetic resonance imaging anterior-posterior and lateral RVOT diameters were smaller in implanted versus nonimplanted patients (18.0 ± 3.6 mm vs. 20.1 ± 3.5 mm; p = 0.005; 18.4 ± 4.3 mm vs. 21.5 ± 3.8 mm; p = 0.002).TPVR in the nRVOT was feasible and safe. However, nearly half the patients presenting for catheterization did not undergo TPV implantation, mainly because of prohibitively large nRVOT size. Improved understanding of magnetic resonance imaging data and availability of larger devices may improve the success rate for nRVOT TPVR.

  View details for PubMedID 30077685

• Mortality prediction after transcatheter treatment of failed bioprosthetic aortic valves utilizing various international scoring systems: Insights from the Valve-in-Valve International Data (VIVID). Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Aziz, M., Simonato, M., Webb, J. G., Abdel-Wahab, M., McElhinney, D., Duncan, A., Tchetche, D., Barbanti, M., Petronio, A. S., Maisano, F., Ribeiro, V. G., Gaia, D. F., Rana, R., Kocka, V., Mathur, M., Wijeysundera, H., Hellig, F., Nissen, H., Bekeredjian, R., Rihal, C., Duffy, S. J., Dvir, D. 2018

  Abstract

  BACKGROUND: Transcatheter Aortic Valve Implantation (TAVI) is commonly used to deploy new bioprosthetic valves inside degenerated surgically implanted aortic valves in high risk patients. The three scoring systems used to assess risk of postprocedural mortality are: Logistic EuroSCORE (LES), EuroSCORE II (ES II), and Society of Thoracic Surgeons (STS).OBJECTIVE: The purpose of this study is to analyze the accuracy of LES, ES II, and STS in estimating all-cause mortality after transcatheter aortic valve-in-valve (ViV) implantations, which was not assessed before.METHODS: Using the Valve-in-Valve International Data (VIVID) registry, a total of 1,550 patients from 110 centers were included. The study compared the observed 30-day overall mortality vs. the respective predicted mortalities calculated by risk scores. The accuracy of prediction models was assessed based on calibration and discrimination.RESULTS: Observed mortality at 30 days was 5.3%, while average expected mortalities by LES, ES II and STS were 29.49 (± 17.2), 14.59 (± 8.6), and 9.61 (± 8.51), respectively. All three risk scores overestimated 30-day mortality with ratios of 0.176 (95% CI 0.138-0.214), 0.342 (95% CI 0.264-0.419), and 0.536 (95% CI 0.421-0.651), respectively. 30-day mortality ROC curves demonstrated that ES II had the largest AUC at 0.722, followed by STS at 0.704, and LES at 0.698.CONCLUSIONS: All three scores overestimated mortality at 30 days with ES II showing the highest predictability compared to LES and STS; and therefore, should be recommended for ViV procedures. There is a need for a dedicated scoring system for patients undergoing ViV interventions.

  View details for PubMedID 30079597

• Postoperative feeding problems in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals undergoing unifocalisation surgery. Cardiology in the young Koth, A. M., Sakarovitch, C., Sidell, D. R., Schultz, L. M., Freccero, A., Rizzuto, S., Hanley, F. L., Asija, R. 2018: 1–4

  Abstract

  BACKGROUND: Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals are at risk for prolonged hospitalisation after unifocalisation. Feeding problems after congenital heart surgery are associated with longer hospital stay. We sought to determine the impact of baseline, intra-operative, and postoperative factors on the need for feeding tube use at the time of discharge.METHODS: We included patients with the aforementioned diagnosis undergoing unifocalisation from ages 3 months to 4 years from 2010 to 2016. We excluded patients with a pre-existing feeding tube. Patients discharged with an enteric tube were included in the feeding tube group. We compared the feeding tube group with the non-feeding-tube group by univariable and multi-variable logistic regression.RESULTS: Of the 56 patients studied, 41% used tube feeding. Median age and weight z-score were similar in the two groups. A chromosome 22q11 deletion was associated with the need for a feeding tube (22q11 deletion in 39% versus 15%, p=0.05). Median cardiopulmonary bypass time in the feeding tube group was longer (335 versus 244 minutes, p=0.04). Prolonged duration of mechanical ventilation was associated with feeding tube use (48 versus 3%, p=0.001). On multi-variable analysis, prolonged mechanical ventilation was associated with feeding tube use (odds ratio 10.2, 95% confidence intervals 1.6; 63.8).CONCLUSION: Among patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals who were feeding by mouth before surgery, prolonged mechanical ventilation after unifocalisation surgery was associated with feeding tube use at discharge. Anticipation of feeding problems in this population and earlier feeding tube placement may reduce hospital length of stay.

  View details for DOI 10.1017/S1047951118001270

  View details for PubMedID 30070195

• Intentional Fracture of Bioprosthetic Valve Frames in Patients Undergoing Valve-in-Valve Transcatheter Pulmonary Valve Replacement. Circulation. Cardiovascular interventions Shahanavaz, S., Asnes, J. D., Grohmann, J., Qureshi, A. M., Rome, J. J., Tanase, D., Crystal, M. A., Latson, L. A., Morray, B. H., Hellenbrand, W., Balzer, D. T., Gewillig, M., Love, J. C., Berdjis, F., Gillespie, M. J., McElhinney, D. B. 2018; 11 (8): e006453

  Abstract

  Percutaneous transcatheter pulmonary valve replacement (TPVR) has good clinical and hemodynamic outcomes in treating dysfunctional bioprosthetic valves (BPV) in the pulmonary position. Valve-in-valve therapy can further decrease the inner diameter (ID), potentially resulting in patient-prosthesis mismatch in patients with smaller BPVs.To evaluate feasibility and outcomes of intentional BPV fracture to enlarge the pulmonary valve orifice with TPVR, 37 patients from 13 centers who underwent TPVR with intended BPV fracture were evaluated. A control cohort (n=70) who underwent valve-in-valve TPVR without attempted fracture was evaluated. BPV was successfully fractured in 28 patients and stretched in 5 while fracture was unsuccessful in 4. A Melody valve was implanted in 25 patients with fractured/stretched frame and a Sapien (XT 3) valve in 8. Among patients whose BPV was fractured/stretched, the final ID was a median of 2 mm larger (0-6.5 mm) than the valve's true ID. The narrowest diameter after TPVR in controls was a median of 2 mm smaller ( P<0.001) than true ID. Right ventricular outflow tract gradient decreased from median 40 to 8 mm Hg in the fracture group. Cases with fracture/stretching were matched 1:1 (weight, true ID) to controls. Post-TPVR peak gradient was lower but not significant (8.3±5.2 versus 11.8±9.2 mm Hg; P=0.070). There were no fracture-related adverse events.Preliminary experience shows intentional fracture of BPV frame can be useful for achieving larger ID and better hemodynamics after valve-in-valve TPVR.

  View details for DOI 10.1161/CIRCINTERVENTIONS.118.006453

  View details for PubMedID 30354783

• Pathological antibody-mediated rejection in pediatric heart transplant recipients: Immunologic risk factors, hemodynamic significance, and outcomes PEDIATRIC TRANSPLANTATION Hollander, S. A., Peng, D. M., Mills, M., Berry, G. J., Fedrigo, M., McElhinney, D. B., Almond, C. S., Rosenthal, D. N. 2018; 22 (5)

  View details for DOI 10.1111/petr.13197

  View details for Web of Science ID 000438730000006

• Safety of ferumoxytol in children undergoing cardiac MRI under general anaesthesia. Cardiology in the young Wise-Faberowski, L., Velasquez, N., Chan, F., Vasanawala, S., McElhinney, D. B., Ramamoorthy, C. 2018; 28 (7): 916–21

  Abstract

  BACKGROUND: Ferumoxytol, an "off-label" contrast agent, allows for better cardiac MRI quality as compared with gadolinium-based contrast agents. However, hypotension has been reported with the use of ferumoxytol for indications other than cardiac MRI. The purpose of our investigation was to evaluate the safety of ferumoxytol in children undergoing general anaesthesia for cardiac MRI.METHODS: Medical records of children undergoing general anaesthesia for cardiac MRI were reviewed. Baseline demographic and medical characteristics, as well as imaging and anaesthetic duration and technique, were collected. The incidence of hypotension or other adverse events', need for vasoactive support, or airway intervention throughout the anaesthetic, was recorded.RESULTS: A total of 95 patients were identified, 61 received ferumoxytol and 34 received gadolinium. There were no significant differences between groups with respect to age, weight, or baseline blood pressure. The incidence of low blood pressure - systolic or mean - after contrast administration did not differ between groups, and there was no difference in sustained hypotension or use of vasopressors between groups. One patient who received ferumoxytol had possible anaphylaxis. The image acquisition time (45 versus 68 min, p=0.002) and anaesthesia duration (100 versus 132 min, p=0.02) were shorter in the ferumoxytol group.CONCLUSION: Transient low blood pressure was common in children undergoing cardiac MRI with anaesthesia, but the incidence of hypotension did not differ between ferumoxytol and gadolinium groups. The use of ferumoxytol was associated with significantly shorter scan time and anaesthesia duration, as well as a decreased need for airway intervention.

  View details for PubMedID 29848399

• Safety of ferumoxytol in children undergoing cardiac MRI under general anaesthesia CARDIOLOGY IN THE YOUNG Wise-Faberowski, L., Velasquez, N., Chan, F., Vasanawala, S., McElhinney, D. B., Ramamoorthy, C. 2018; 28 (7): 916–21

  View details for DOI 10.1017/S1047951118000306

  View details for Web of Science ID 000434245900004

• Direct physiologic assessment of anomalous aortic origin of a coronary artery: Enhanced diagnostics or illusion of insight? Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions McElhinney, D. B. 2018; 92 (1): 76-77

  Abstract

  Fractional flow reserve and intravascular ultrasound evaluation are technically feasible in patients with congenitally abnormal coronary arteries arising from the wrong aortic sinus, including those with a slitlike orifice and presumed intramural course. Incorporation of fractional flow reserve and intravascular ultrasound into the evaluation of adults with anomalous coronary artery origins will provide additional data and may be useful in risk assessment, although these data should be interpreted with caution, as the validity of fractional flow reserve findings in this population has not been established. The applicability of fractional flow reserve thresholds for acute coronary syndromes to patients with anomalous coronary artery origins is unknown. Further study will be needed to determine how best to incorporate fractional flow reserve data in the evaluation and management of these patients.

  View details for DOI 10.1002/ccd.27695

  View details for PubMedID 30917218

• Circulation: Cardiovascular Interventions: The First 10 Years CIRCULATION-CARDIOVASCULAR INTERVENTIONS Faxon, D. P., Leopold, J. A., Abbott, J., McElhinney, D. B., Williams, D. O. 2018; 11 (6): e006901

  View details for PubMedID 29895606

• Pathological antibody-mediated rejection in pediatric heart transplant recipients: Immunologic risk factors, hemodynamic significance, and outcomes. Pediatric transplantation Hollander, S. A., Peng, D. M., Mills, M., Berry, G. J., Fedrigo, M., McElhinney, D. B., Almond, C. S., Rosenthal, D. N. 2018: e13197

  Abstract

  Biopsy-diagnosed pAMR has been observed in over half of pediatric HT recipients within 6years of transplantation. We report the incidence and outcomes of pAMR at our center. All endomyocardial biopsies for all HT recipients transplanted between 2010 and 2015 were reviewed and classified using contemporary ISHLT guidelines. Graft dysfunction was defined as a qualitative decrement in systolic function by echocardiogram or an increase of ≥3mmHg in atrial filling pressure by direct measurement. Among 96 patients, pAMR2 occurred in 7 (7%) over a median follow-up period of 3.1years, while no cases of pAMR3 occurred. A history of CHD, DSA at transplant, and elevated filling pressures were associated with pAMR2. Five-sixths (83%) of patients developed new C1q+ DSA at the time of pAMR diagnosis. There was a trend toward reduced survival, with 43% of patients dying within 2.3years of pAMR diagnosis.

  View details for PubMedID 29729067

• Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia. The Journal of thoracic and cardiovascular surgery Koth, A. M., Kwiatkowski, D. M., Lim, T. R., Bauser-Heaton, H., Asija, R., McElhinney, D. B., Hanley, F. L., Krawczeski, C. D. 2018

  Abstract

  BACKGROUND: We set out to determine whether patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCA) are at risk for elevated dead space ventilation fraction (VD/VT), and whether this is associated with prolonged mechanical ventilation. We hypothesized that elevated VD/VT (>20%) in the first 24hours after unifocalization surgery is associated with increased risk for prolonged mechanical ventilation (>7days).METHODS: All patients with TOF/PA/MAPCA undergoing unifocalization surgery between January 2003 and December 2015 were included in this study. Average VD/VT was calculated over the first 24hours after surgery. Demographic and surgical data were collected. Outcome data included duration of mechanical ventilation. Patients were separated into 2 groups: elevated VD/VT and normal DVSF. Groups were compared using the Student t test, Wilcoxon rank-sum test, and chi2 test. Univariable and multivariable regression analyses were performed with VD/VT as a continuous variable to test for association.RESULTS: Of the 265 included patients, 127 (48%) had an elevated VD/VT. The 2 groups did not differ significantly in any demographic characteristic. Patients with an elevated VD/VT had longer cardiopulmonary bypass times (P=.03), were more likely to have delayed sternal closure, and more likely to have prolonged respiratory failure (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P=.007). The percent VD/VT was associated with duration of mechanical ventilation in univariable (P<.001) and multivariable (P<.001) regression analyses when controlled for age, weight and bypass time.CONCLUSIONS: Elevated postoperative VD/VT is associated with prolonged mechanical ventilation in patients with TOF/PA/MAPCA following unifocalization. Elevated postoperative VD/VT may be an early indicator of patients who will require prolonged duration of mechanical ventilation, allowing optimization of medical management to promote better outcomes.

  View details for PubMedID 29884495

• Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bauser-Heaton, H., Borquez, A., Asija, R., Wise-Faberowski, L., Zhang, Y., Downey, L., Perry, S. B., Koth, A., Peng, L. F., Algaze, C. A., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1696–1707

  Abstract

  Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.

  View details for PubMedID 29352588

• Transcatheter pulmonary valve replacement using the melody valve for treatment of dysfunctional surgical bioprostheses: A multicenter study Cabalka, A. K., Asnes, J. D., Balzer, D. T., Cheatham, J. P., Gillespie, M. J., Jones, T. K., Justino, H., Kim, D. W., Lung, T., Turner, D. R., McElhinney, D. B. MOSBY-ELSEVIER. 2018: 1712-+

  Abstract

  Stented bioprosthetic valves (BPVs) are commonly used for surgical pulmonary valve (PV) replacement in postoperative congenital heart disease, but develop structural failure in a time-related fashion. The Melody transcatheter PV (TPV) (Medtronic, Minneapolis, Minn) has been used to treat BPV dysfunction, but there have been few studies in this population.We performed a retrospective, multicenter study to evaluate Melody valve function in patients who underwent TPV replacement (TPVR) into a dysfunctional pulmonary BPV.One hundred patients who underwent TPVR at 10 centers between January 2010 and June 2015 were enrolled. The median patient age was 22 years (range, 5-79 years), and 32 patients were age <18 years. The underlying diagnosis was tetralogy of Fallot in 80 patients, and moderate or severe pulmonary regurgitation (PR) was present in 84%. The TPV was implanted into various types of BPVs, with a median size of 23 mm (range, 19-33 mm). At hospital discharge, PR was mild or less in all but 1 patient, and the mean Doppler right ventricular outflow tract (RVOT) gradient was reduced from a mean of 29.3 ± 12.0 mm Hg to 16.2 ± 6.9 mm Hg (median, 29 mm Hg to 16 mm Hg; P < .001). During follow-up (median, 12.4 months), no patients underwent reintervention on the TPV. Endocarditis was diagnosed in 1 patient who was managed medically without intervention. The mean RVOT gradient at the most recent follow-up was ≤35 mm Hg in all patients, and was similar to that at early postimplantation. PR was more than mild in only 1 patient. Hemodynamic outcomes did not differ between patients with small BPVs (≤23 mm) and those with large BPVs (≥25 mm).TPVR restores competence and relieves the obstruction of dysfunctional surgical BPVs, with excellent early results in both small and large BPVs, highlighting the potential for TPVR to extend the life of existing BPVs in adults and children. Collaboration between surgeons and cardiologists is important to determine the optimal lifetime management, combining surgical PV replacement and TPVR in this population.

  View details for PubMedID 29395214

• Readmissionsfor Children Hospitalized with Heart Failure: Are Some ReadmissionsPreventable? Knoll, C., Dykes, J., Shetty, R., Liu, E., Rosenthal, D., Chen, S., McElhinney, D., Almond, C. ELSEVIER SCIENCE INC. 2018: S389

  View details for Web of Science ID 000430727300393

• Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sandeep, N., Punn, R., Balasubramanian, S., Smith, S. N., Reinhartz, O., Zhang, Y., Wright, G. E., Peng, L. F., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1747-+

  Abstract

  Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling.This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed.On competing risk analysis, survival over time was better in the valved conduit group (P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn.In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function.

  View details for PubMedID 29223842

• Transcatheter Pulmonary Valve Replacement With the Melody Valve in Small Diameter Expandable Right Ventricular Outflow Tract Conduits JACC-CARDIOVASCULAR INTERVENTIONS Shahanavaz, S., Qureshi, A. M., Levi, D. S., Boudjemline, Y., Peng, L. F., Martin, M., Bauser-Heaton, H., Keeshan, B., Asnes, J. D., Jones, T. K., Justino, H., Aboulhosn, J. A., Gray, R. G., Nguyen, H., Balzer, D. T., McElhinney, D. B. 2018; 11 (6): 554–64

  Abstract

  This study sought to evaluate the safety, feasibility, and outcomes of transcatheter pulmonary valve replacement (TPVR) in conduits ≤16 mm in diameter.The Melody valve (Medtronic, Minneapolis, Minnesota) is approved for the treatment of dysfunctional right ventricular outflow tract (RVOT) conduits ≥16 mm in diameter at the time of implant. Limited data are available regarding the use of this device in smaller conduits.The study retrospectively evaluated patients from 9 centers who underwent percutaneous TPVR into a conduit that was ≤16 mm in diameter at the time of implant, and reported procedural characteristics and outcomes.A total of 140 patients were included and 117 patients (78%; median age and weight 11 years of age and 35 kg, respectively) underwent successful TPVR. The median original conduit diameter was 15 (range: 9 to 16) mm, and the median narrowest conduit diameter was 11 (range: 4 to 23) mm. Conduits were enlarged to a median diameter of 19 mm (29% larger than the implanted diameter), with no difference between conduits. There was significant hemodynamic improvement post-implant, with a residual peak RVOT pressure gradient of 7 mm Hg (p < 0.001) and no significant pulmonary regurgitation. During a median follow-up of 2.0 years, freedom from RVOT reintervention was 97% and 89% at 2 and 4 years, respectively, and there were no deaths and 5 cases of endocarditis (incidence rate 2.0% per patient-year).In this preliminary experience, TPVR with the Melody valve into expandable small diameter conduits was feasible and safe, with favorable early and long-term procedural and hemodynamic outcomes.

  View details for PubMedID 29566801

• Branch Pulmonary Artery Valve Implantation Reduces Pulmonary Regurgitation and Improves Right Ventricular Size/Function in Patients With Large Right Ventricular Outflow Tracts JACC-CARDIOVASCULAR INTERVENTIONS Qureshi, A. M., Bansal, N., McElhinney, D. B., Boudjemline, Y., Forbes, T. J., Maschietto, N., Shahanavaz, S., Cheatham, J. P., Krasuski, R., Lamers, L., Chessa, M., Morray, B. H., Goldstein, B. H., Noel, C. V., Wang, Y., Gillespie, M. J. 2018; 11 (6): 541–50

  Abstract

  The authors sought to assess the intermediate-term effects of percutaneous placed valves in the branch pulmonary artery (PA) position.Most patients with large right ventricular outflow tracts (RVOTs) are excluded from available percutaneous pulmonary valve options. In some of these patients, percutaneous branch PA valve implantation may be feasible. The longer-term effects of valves in the branch PA position is unknown.Retrospective data were collected on patients with significant pulmonary regurgitation who had a percutaneous branch PA valve attempted.Percutaneous branch PA valve implantation was attempted in 34 patients (18 bilateral and 16 unilateral). One-half of the patients were in New York Heart Association (NHYA) functional class III or IV pre-implantation. There were 2 failed attempts and 6 procedural complications. At follow-up, only 1 patient had more than mild valvar regurgitation. The right ventricular end-diastolic volume index decreased from 147 (range: 103 to 478) ml/m2 to 101 (range: 76 to 429) ml/m2, p < 0.01 (n = 16), and the right ventricular end-systolic volume index decreased from 88.5 (range: 41 to 387) ml/m2 to 55.5 (range: 40.2 to 347) ml/m2, p < 0.01 (n = 13). There were 5 late deaths. At a median follow-up of 2 years, all other patients were in NYHA functional class I or II.Percutaneous branch PA valve implantation results in a reduction in right ventricular volume with clinical benefit in the intermediate term. Until percutaneous valve technology for large RVOTs is refined and more widely available, branch PA valve implantation remains an option for select patients.

  View details for PubMedID 29566799

• QUALITY OF INR CONTROL IN A CENTRALIZED PHARMACIST-LED PEDIATRIC CARDIOLOGY WARFARIN MANAGEMENT PROGRAM Lee, J., Murray, J., Wilkens, S., Zhang, Y., Nishioka, F., Rosenthal, D., Roth, S., Murphy, D., McElhinney, D., Almond, C. ELSEVIER SCIENCE INC. 2018: 609

  View details for DOI 10.1016/S0735-1097(18)31150-1

  View details for Web of Science ID 000429659701459

• Anesthetic Management During Surgery for Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY Quinonez, Z. A., Downey, L., Abbasi, R. K., Kuan, C., Asija, R., McElhinney, D. B., Hanley, F. L., Mainwaring, R. D., Wise-Faberowski, L. 2018; 9 (2): 236–41

  Abstract

  Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures. Herein, we describe our anesthetic management based on our own institution's surgical approach at Lucile Packard Children's Hospital/Stanford University.

  View details for PubMedID 29544416

• Percutaneous transcatheter pulmonary valve replacement in children weighing less than 20 kg CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Martin, M., Shahanavaz, S., Peng, L. F., Asnes, J. D., Riley, M., Hellenbrand, W. E., Balzer, D. T., Gray, R. G., McElhinney, D. B. 2018; 91 (3): 485–94

  Abstract

  Since FDA approval of the Melody valve, transcatheter pulmonary valve replacement (TPVR) has been offered to an expanding population. Limited data exist regarding the safety and feasibility of TPVR in smaller patients.All patients weighing <20 kg who underwent catheterization for percutaneous TPVR at four centers were reviewed.Of the 51 patients reviewed, 43 (84%) had successful valve implantation and 8 (16%) were found not to be candidates due to access veins too small (n = 3), coronary artery/aortic root compression (n = 3), and RVOT too large (n = 2). The 43 patients who underwent successful percutaneous TPVR had a median age and weight of 5.8 years (3.3-10) and 17.7 kg (13.5-19.8), and most had tetralogy of Fallot (TOF). TPVR was performed via femoral vein (FV) access in 24 patients and internal jugular vein (IJV) access in 19 patients. Median weight in patients with IJV implant was 1.1 kg lower than those who underwent transfemoral implant (17.0 vs. 18.1 kg, P = 0.05). There were four adverse events: one iliac vein injury, one contained MPA tear, and two patients with post-procedure femoral vein bleeding. All patients were alive at recent follow-up, a median of 2.0 years (0.1-6.0) after TPVR. There was excellent valve function with mean Doppler gradients of 3-20 mm Hg, and trivial or mild pulmonary regurgitation in all but one patient. There were no RVOT reinterventions and no cases of endocarditis.Percutaneous TPVR can be safely performed in patients <20 kg. The procedure frequently requires IJV access, and access site/bleeding complications may be more common in this cohort.

  View details for PubMedID 29193671

• Outcomes of Transcatheter Tricuspid Valve-in-Valve Implantation in Patients With Ebstein Anomaly AMERICAN JOURNAL OF CARDIOLOGY Taggart, N. W., Cabalka, A. K., Eicken, A., Aboulhosn, J. A., Thomson, J. R., Whisenant, B., Bocks, M. L., Schubert, S., Jones, T. K., Asnes, J. D., Fagan, T. E., Meadows, J., Hoyer, M., Martin, M. H., Ing, F. F., Turner, D. R., Latib, A., Tzifa, A., Windecker, S., Goldstein, B. H., Delaney, J. W., Kuo, J. A., Foerster, S., Gillespie, M., Butera, G., Shahanavaz, S., Horlick, E., Boudjemline, Y., Dvir, D., McElhinney, D. B., VIVID Registry 2018; 121 (2): 262–68

  Abstract

  We sought to describe the acute results and short- to medium-term durability of transcatheter tricuspid valve-in-valve (TVIV) implantation within surgical bioprostheses among patients with Ebstein anomaly (EA). Cases were identified from a voluntary, multicenter, international registry of 29 institutions that perform TVIV. Demographic, clinical, procedural, and follow-up data were analyzed. Eighty-one patients with EA underwent TVIV from 2008 to 2016. Thirty-four patients (42%) were New York Heart Association (NYHA) class 3/4 at time of TVIV. The most common indication for TVIV was the presence of moderate or severe tricuspid regurgitation (40%). Most patients received a Melody valve (64%). TVIV was ultimately successful in all patients, and there was no procedural mortality. Four patients (5%) developed acute valve thrombosis, 4 patients (5%) developed endocarditis, and 9 patients (11%) developed valve dysfunction not related to thrombosis or endocarditis. Eight patients (10%) underwent reintervention (2 transcatheter, 6 surgical) due to thrombosis (3), endocarditis (2), other valve dysfunction (2), and patient-prosthesis mismatch without valve dysfunction (1). Among 69 patients who were alive without reintervention at latest follow-up, 96% of those with NYHA status reported were class 1/2, a significant improvement from baseline (62% NYHA class 1/2, p <0.001). In conclusion, transcatheter TVIV offers a low-risk, minimally invasive alternative to surgical tricuspid valve re-replacement in patients with EA and a failing tricuspid valve bioprosthesis.

  View details for PubMedID 29153244

• Assessing Statewide All-Cause Future One-Year Mortality: Prospective Study With Implications for Quality of Life, Resource Utilization, and Medical Futility. Journal of medical Internet research Guo, Y. n., Zheng, G. n., Fu, T. n., Hao, S. n., Ye, C. n., Zheng, L. n., Liu, M. n., Xia, M. n., Jin, B. n., Zhu, C. n., Wang, O. n., Wu, Q. n., Culver, D. S., Alfreds, S. T., Stearns, F. n., Kanov, L. n., Bhatia, A. n., Sylvester, K. G., Widen, E. n., McElhinney, D. B., Ling, X. B. 2018; 20 (6): e10311

  Abstract

  For many elderly patients, a disproportionate amount of health care resources and expenditures is spent during the last year of life, despite the discomfort and reduced quality of life associated with many aggressive medical approaches. However, few prognostic tools have focused on predicting all-cause 1-year mortality among elderly patients at a statewide level, an issue that has implications for improving quality of life while distributing scarce resources fairly.Using data from a statewide elderly population (aged ≥65 years), we sought to prospectively validate an algorithm to identify patients at risk for dying in the next year for the purpose of minimizing decision uncertainty, improving quality of life, and reducing futile treatment.Analysis was performed using electronic medical records from the Health Information Exchange in the state of Maine, which covered records of nearly 95% of the statewide population. The model was developed from 125,896 patients aged at least 65 years who were discharged from any care facility in the Health Information Exchange network from September 5, 2013, to September 4, 2015. Validation was conducted using 153,199 patients with same inclusion and exclusion criteria from September 5, 2014, to September 4, 2016. Patients were stratified into risk groups. The association between all-cause 1-year mortality and risk factors was screened by chi-squared test and manually reviewed by 2 clinicians. We calculated risk scores for individual patients using a gradient tree-based boost algorithm, which measured the probability of mortality within the next year based on the preceding 1-year clinical profile.The development sample included 125,896 patients (72,572 women, 57.64%; mean 74.2 [SD 7.7] years). The final validation cohort included 153,199 patients (88,177 women, 57.56%; mean 74.3 [SD 7.8] years). The c-statistic for discrimination was 0.96 (95% CI 0.93-0.98) in the development group and 0.91 (95% CI 0.90-0.94) in the validation cohort. The mortality was 0.99% in the low-risk group, 16.75% in the intermediate-risk group, and 72.12% in the high-risk group. A total of 99 independent risk factors (n=99) for mortality were identified (reported as odds ratios; 95% CI). Age was on the top of list (1.41; 1.06-1.48); congestive heart failure (20.90; 15.41-28.08) and different tumor sites were also recognized as driving risk factors, such as cancer of the ovaries (14.42; 2.24-53.04), colon (14.07; 10.08-19.08), and stomach (13.64; 3.26-86.57). Disparities were also found in patients' social determinants like respiratory hazard index (1.24; 0.92-1.40) and unemployment rate (1.18; 0.98-1.24). Among high-risk patients who expired in our dataset, cerebrovascular accident, amputation, and type 1 diabetes were the top 3 diseases in terms of average cost in the last year of life.Our study prospectively validated an accurate 1-year risk prediction model and stratification for the elderly population (≥65 years) at risk of mortality with statewide electronic medical record datasets. It should be a valuable adjunct for helping patients to make better quality-of-life choices and alerting care givers to target high-risk elderly for appropriate care and discussions, thus cutting back on futile treatment.

  View details for PubMedID 29866643

• Classic-Pattern Dyssynchrony in Adolescents and Adults With a Fontan Circulation. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Rösner, A. n., Khalapyan, T. n., Dalen, H. n., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 31 (2): 211–19

  Abstract

  Previous studies have suggested the presence of dyssynchrony in the functionally single ventricle. The aim of this study was to investigate the presence of classic-pattern dyssynchrony (CPD), characterized by typical early and late deformation of opposite walls, and its relation to QRS duration and myocardial function in patients with single-ventricle physiology after Fontan palliation.In a retrospective cross-sectional study, 101 adolescent and adult patients with single-ventricle physiology after the Fontan procedure were investigated. Strain curves were visually assessed for the presence of CPD. Systolic and diastolic function were assessed using echocardiography.One hundred one patients were included, with varying anatomic morphology: two sizable ventricular components (n = 21), right dominant (n = 21), left dominant (n = 49), and undefined anatomy (n = 10). Fifteen of 101 Fontan patients had CPD. Forty-three percent of patients with two sizable ventricular masses displayed CPD, mostly with prolonged QRS, while the number of patients with CPD with right-dominant (9%) and left-dominant (6%) morphology was significantly lower (P = .016). Those with CPD displayed significantly (P < .05) larger QRS widths (142 ± 22 vs 112 ± 24 msec), lower ejection fractions (31 ± 14% vs 45 ± 14%), lower global early diastolic strain rates (0.7 ± 0.5 vs 1.2 ± 0.8 sec-1), and global systolic circumferential (-10 ± 5% vs -16 ± 7%) and longitudinal (-9 ± 5% vs -14 ± 5%) strain, respectively.CPD is present in a proportion of adolescent and adult patients after Fontan palliation. The presence of CPD is associated with reduced systolic and diastolic function compared with Fontan patients without CPD. Because the presence of CPD appears to be a promising predictor for response to cardiac resynchronization therapy in patients with biventricular circulation, these findings may have important potential for prospective evaluation of cardiac resynchronization therapy in patients with univentricular circulation.

  View details for PubMedID 29229494

• Prediction of Incident Hypertension Within the Next Year: Prospective Study Using Statewide Electronic Health Records and Machine Learning. Journal of medical Internet research Ye, C. n., Fu, T. n., Hao, S. n., Zhang, Y. n., Wang, O. n., Jin, B. n., Xia, M. n., Liu, M. n., Zhou, X. n., Wu, Q. n., Guo, Y. n., Zhu, C. n., Li, Y. M., Culver, D. S., Alfreds, S. T., Stearns, F. n., Sylvester, K. G., Widen, E. n., McElhinney, D. n., Ling, X. n. 2018; 20 (1): e22

  Abstract

  As a high-prevalence health condition, hypertension is clinically costly, difficult to manage, and often leads to severe and life-threatening diseases such as cardiovascular disease (CVD) and stroke.The aim of this study was to develop and validate prospectively a risk prediction model of incident essential hypertension within the following year.Data from individual patient electronic health records (EHRs) were extracted from the Maine Health Information Exchange network. Retrospective (N=823,627, calendar year 2013) and prospective (N=680,810, calendar year 2014) cohorts were formed. A machine learning algorithm, XGBoost, was adopted in the process of feature selection and model building. It generated an ensemble of classification trees and assigned a final predictive risk score to each individual.The 1-year incident hypertension risk model attained areas under the curve (AUCs) of 0.917 and 0.870 in the retrospective and prospective cohorts, respectively. Risk scores were calculated and stratified into five risk categories, with 4526 out of 381,544 patients (1.19%) in the lowest risk category (score 0-0.05) and 21,050 out of 41,329 patients (50.93%) in the highest risk category (score 0.4-1) receiving a diagnosis of incident hypertension in the following 1 year. Type 2 diabetes, lipid disorders, CVDs, mental illness, clinical utilization indicators, and socioeconomic determinants were recognized as driving or associated features of incident essential hypertension. The very high risk population mainly comprised elderly (age>50 years) individuals with multiple chronic conditions, especially those receiving medications for mental disorders. Disparities were also found in social determinants, including some community-level factors associated with higher risk and others that were protective against hypertension.With statewide EHR datasets, our study prospectively validated an accurate 1-year risk prediction model for incident essential hypertension. Our real-time predictive analytic model has been deployed in the state of Maine, providing implications in interventions for hypertension and related diseases and hopefully enhancing hypertension care.

  View details for PubMedID 29382633

• Relationships Among Conduit Type, Pre-Stenting, and Outcomes in Patients Undergoing Transcatheter Pulmonary Valve Replacement in the Prospective North American and European Melody Valve Trials JACC-CARDIOVASCULAR INTERVENTIONS Cabalka, A. K., Hellenbrand, W. E., Eicken, A., Kreutzer, J., Gray, R. G., Bergersen, L., Berger, F., Armstrong, A. K., Cheatham, J. P., Zahn, E. M., McElhinney, D. B. 2017; 10 (17): 1746–59

  Abstract

  This study sought to evaluate the incidence of and risk factors for conduit and stent-related outcomes following transcatheter pulmonary valve replacement (TPVR).Stent fracture (SF) and right ventricular outflow tract (RVOT) reintervention are among the most important adverse outcomes after TPVR using the Melody valve (Medtronic, Minneapolis, Minnesota). The conduit environment and conduit preparation practices vary among patients who undergo TPVR.Data from 3 prospective Melody valve multicenter studies were pooled and analyzed. All patients who had successful implant of a Melody valve that was present at hospital discharge comprised the study cohort; patients who had TPVR into a stentless conduit comprised the analysis cohort. SF was diagnosed using protocol-specified or clinical fluoroscopy or radiography, and classified as major or minor.Of 358 patients who underwent catheterization with intent to perform TPVR, 309 were discharged with the Melody valve in place (study cohort) of which 251 patients had TPVR into a stentless conduit (analysis cohort). Median follow-up was 5 years. New pre-stents were placed in 68% of patients with a stentless conduit, and 22% received multiple pre-stents. At 3 years, freedom from any SF and major SF was 74 ± 3% and 85 ± 2%, respectively, and freedom from RVOT reintervention was 85 ± 2%. New pre-stents were associated with longer freedom from SF and RVOT reintervention than was no pre-stent.Risks of SF and reintervention after TPVR with a Melody valve were reduced by implantation of pre-stents, which has become standard practice. This study supports pre-stenting as an important component of TPVR therapy.

  View details for DOI 10.1016/j.jcin.2017.05.022

  View details for Web of Science ID 000409098800009

  View details for PubMedID 28823778

• When Device Failure Translates to Therapeutic Efficacy: Intentional Fracture of Bioprosthetic Valve Rings as an Adjunctive Technique in Transcatheter Valve-in-Valve Replacement. Circulation. Cardiovascular interventions McElhinney, D. B. 2017; 10 (7)

  View details for PubMedID 28698294

• When Device Failure Translates to Therapeutic Efficacy Intentional Fracture of Bioprosthetic Valve Rings as an Adjunctive Technique in Transcatheter Valve-in-Valve Replacement CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2017; 10 (7)

  View details for DOI 10.1161/CIRCINTERVENTIONS.117.005662

  View details for Web of Science ID 000405661400009

• Multicenter Experience Evaluating Transcatheter Pulmonary Valve Replacement in Bovine Jugular Vein (Contegra) Right Ventricle to Pulmonary Artery Conduits. Circulation. Cardiovascular interventions Morray, B. H., McElhinney, D. B., Boudjemline, Y., Gewillig, M., Kim, D. W., Grant, E. K., Bocks, M. L., Martin, M. H., Armstrong, A. K., Berman, D., Danon, S., Hoyer, M., Delaney, J. W., Justino, H., Qureshi, A. M., Meadows, J. J., Jones, T. K. 2017; 10 (6)

  Abstract

  BACKGROUND: Follow-up of transcatheter pulmonary valve replacement (TPVR) with the Melody valve has demonstrated good short-term and long-term outcomes, but there are no published studies focused on valve performance in the Contegra bovine jugular vein conduit.METHODS AND RESULTS: This is a retrospective, multicenter study of the short- and intermediate-term outcomes of Melody TPVR within the Contegra conduit in the right ventricle to pulmonary artery position. Data from 13 centers were included in the analysis. During the study period, 136 patients underwent 139 catheterizations for attempted Melody TPVR with a median follow-up of 3 years (1 day to 9.1 years). Of the 136 patients, 117 underwent successful Melody TPVR. Two patients underwent a second Melody TPVR. The majority of patients underwent placement of ≥1 stents before transcatheter pulmonary valve implantation. There was a significant reduction in peak conduit pressure gradient acutely after transcatheter pulmonary valve implantation (39 versus 10 mmHg; P<0.001). At most recent follow-up, the maximum pulmonary valve gradient by echocardiogram remained significantly reduced relative to prevalve implant measurements (65.9 versus 27.3 mmHg; P<0.001). The incidence of Melody transcatheter pulmonary valve stent fracture (3.4%) and infectious endocarditis (4.3%) were both low. Serious adverse events occurred in 3 patients.CONCLUSIONS: Melody TPVR in Contegra conduits is safe and effective and can be performed in a wide range of conduit sizes with preserved valve function and low incidence of stent fracture and endocarditis.

  View details for PubMedID 28600328

• Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals. Pediatric pulmonology Sidell, D. R., Koth, A. M., Bauser-Heaton, H., McElhinney, D. B., Wise-Faberowski, L., Tracy, M. C., Hanley, F. L., Asija, R. 2017

  Abstract

  Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy.Retrospective case-control.All patients with TOF/PA/MAPCAs who underwent unifocalization surgery from September 2005 through March 2016 were included. Patients who underwent bronchoscopy in the perioperative period were compared to a randomly selected cohort of 172 control patients who underwent unifocalization without bronchoscopy during the study period.Forty-three children underwent perioperative bronchoscopy at a median of 9 days postoperatively. Baseline demographics were similar in bronchoscopy patients and controls. Patients who underwent bronchoscopy were more likely to have a chromosome 22q11 deletion and were more likely have undergone unifocalization surgery without intracardiac repair. These patients had a longer duration of mechanical ventilation, ICU duration, and length of hospitalization. Abnormalities were detected on bronchoscopy in 35 patients (81%), and 20 (35%) of bronchoscopy patients underwent a postoperative intervention related to abnormalities identified on bronchoscopy.Bronchoscopy is a useful therapeutic and diagnostic instrument for children undergoing unifocalization surgery, capable of identifying abnormalities leading to an additional intervention in over one third of patients. Special attention should be given to children with a 22q11 deletion to expedite diagnosis and intervention for possible airway complications.

  View details for DOI 10.1002/ppul.23732

  View details for PubMedID 28504356

• Cerebral Oxygen Saturation in Children With Congenital Heart Disease and Chronic Hypoxemia. Anesthesia and analgesia Kussman, B. D., Laussen, P. C., Benni, P. B., McGowan, F. X., McElhinney, D. B. 2017

  Abstract

  Increased hemoglobin (Hb) concentration accompanying hypoxemia is a compensatory response to maintain tissue oxygen delivery. Near infrared spectroscopy (NIRS) is used clinically to detect abnormalities in the balance of cerebral tissue oxygen delivery and consumption, including in children with congenital heart disease (CHD). Although NIRS-measured cerebral tissue O2 saturation (ScO2) correlates with arterial oxygen saturation (SaO2), jugular bulb O2 saturation (SjbO2), and Hb, little data exist on the interplay between these factors and cerebral O2 extraction (COE). This study investigated the associations of ScO2 and ΔSaO2-ScO2 with SaO2 and Hb and verified the normal range of ScO2 in children with CHD.Children undergoing cardiac catheterization for CHD were enrolled in a calibration and validation study of the FORE-SIGHT NIRS monitor. Two pairs of simultaneous arterial and jugular bulb samples were drawn for co-oximetry, calculation of a reference ScO2 (REF CX), and estimation of COE. Pearson correlation and linear regression were used to determine relationships between O2 saturation parameters and Hb. Data were also analyzed according to diagnostic group defined as acyanotic (SaO2 ≥ 90%) and cyanotic (SaO2 < 90%).Of 65 children studied, acceptable jugular bulb samples (SjbO2 absolute difference between samples ≤10%) were obtained in 57 (88%). The ΔSaO2-SjbO2, ΔSaO2-ScO2, and ΔSaO2-REF CX were positively correlated with SaO2 and negatively correlated with Hb (all P < .001). Although by diagnostic group ScO2 differed statistically (P = .002), values in the cyanotic patients were within the range considered normal (69% ± 6%). COE estimated by the difference between arterial and jugular bulb O2 content (ΔCaO2-CjbO2, mL O2/100 mL) was not different for cyanotic and acyanotic patients (P = .10), but estimates using ΔSaO2-SjbO2, ΔSaO2-ScO2, or ΔSaO2-ScO2/SaO2 were significantly different between the cyanotic and acyanotic children (P < .001).Children with adequately compensated chronic hypoxemia appear to have ScO2 values within the normal range. The ΔSaO2-ScO2 is inversely related to Hb, with the implication that in the presence of reduced Hb, particularly if coupled with a decreased cardiac output, the ScO2 can fall to values associated with brain injury in laboratory studies.

  View details for DOI 10.1213/ANE.0000000000002073

  View details for PubMedID 28514318

• Transcatheter interventions in adults with congenital heart disease: Surveys from the Society for Cardiovascular Angiography and Interventions to identify current patterns of care and perception on training requirements. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Wadia, S. K., Accavitti, M. J., Morgan, G. J., Kenny, D., Hijazi, Z. M., Jones, T. K., Cabalka, A. K., McElhinney, D. B., Kavinsky, C. J. 2017

  Abstract

  Interventional catheterization is central to the care of Adults with Congenital Heart Disease (ACHD). Current standards for care provision and training in ACHD intervention are lacking. We sought to examine trends in current practice and training among interventionalists.We analyzed the results of two separate international surveys in June 2016. One was sent to all active members from the Society of Cardiovascular Angiography and Interventions (SCAI) who self-identified Structural Heart Disease or Congenital Heart Disease as a practice area. The second survey was conducted through the Pediatric Interventional Early Career Society (PICES) aimed at pediatric and adult congenital interventionalists in their first seven years after training. The total survey sample sizes were 1,535 and 112, respectively.Response rates for the SCAI and PICES surveys were 15% (237/1,535) and 75% (84/112), respectively. Most respondents (74%) worked at institutions with pediatric and adult facilities in proximity (major medical centers). While 75% of SCAI respondents believed complex transcatheter procedures should be performed by ACHD-trained interventionalists or multidisciplinary teams, only 32% reported such care is being provided at the present time. Most pediatric and adult cardiologists surveyed (73%) do not believe current interventional fellowships provide adequate training for proficiency in ACHD interventions.ACHD management remains underdeveloped in relative terms, particularly in the United States. Significant gaps in interventional standards of practice and future training needs were recognized by this study. Our survey should serve as an impetus to establish training guidelines for physicians who seek to perform ACHD interventions.

  View details for DOI 10.1002/ccd.27151

  View details for PubMedID 28493591

• Cost Effectiveness of General Anesthesia in Children for Computerized Tomography Wise-Faberowski, L., Velasquez, N., Aktay, R., McElhinney, D., Chan, F. LIPPINCOTT WILLIAMS & WILKINS. 2017: 895

  View details for Web of Science ID 000412683000454

• Small and Preterm Infants: The Shrinking Frontier of Transcatheter Patent Ductus Arteriosus Closure CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2017; 89 (6): 1066–68

  View details for DOI 10.1002/ccd.27076

  View details for Web of Science ID 000400984400027

  View details for PubMedID 28432825

• Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation. JACC. Cardiovascular interventions Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., Pabst von Ohain, J., McElhinney, D. B., Hager, A., Kühn, A., Eicken, A. 2017; 10 (7): 701-708

  Abstract

  This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI).PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation.In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR.The degree of TR improved in 83% of the patients. In our patients (n = 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months.PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI.

  View details for DOI 10.1016/j.jcin.2017.01.036

  View details for PubMedID 28385408

• Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation JACC-CARDIOVASCULAR INTERVENTIONS Tanase, D., Ewert, P., Georgiev, S., Meierhofer, C., von Ohain, J. P., McElhinney, D. B., Hager, A., Kuehn, A., Eicken, A. 2017; 10 (7): 701-708

  Abstract

  This study sought to investigate the impact of tricuspid regurgitation (TR) on right ventricular function after percutaneous pulmonary valve implantation (PPVI).PPVI provides a less invasive alternative to surgery in patients with right ventricular-to-pulmonary artery (RV-PA) conduit dysfunction. Recovery of the right ventricle has been described after PPVI for patients with pulmonary stenosis and for those with pulmonary regurgitation. Additional TR enforces RV dysfunction by supplemental volume overload. Limited data are available on the potential of the right ventricle to recover in such a specific hemodynamic situation.In a matched cohort study, we compared patients who underwent PPVI with additional TR with those without TR.The degree of TR improved in 83% of the patients. In our patients (n = 36) exercise capacity and right ventricular volume index improved similarly 6 months after PPVI in patients with and without important TR. None of them had significant TR in the long-term follow-up of median 78 months.PPVI improves not only RV-PA-conduit dysfunction, but also concomitant TR. In patients with a dysfunctional RV-PA conduit and TR, the decision whether to fix TR should be postponed after PPVI.

  View details for DOI 10.1016/j.jcin.2017.01.036

  View details for Web of Science ID 000401135000013

• Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients CIRCULATION-CARDIOVASCULAR INTERVENTIONS Bauser-Heaton, H., Borquez, A., Han, B., Ladd, M., Asija, R., Downey, L., Koth, A., Algaze, C. A., Wise-Faberowski, L., Perry, S. B., Shin, A., Peng, L. F., Hanley, F. L., McElhinney, D. B. 2017; 10 (4)

  Abstract

  Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

  View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

  View details for PubMedID 28356265

• Impact of Left Ventricular Assist Device on End-Organ Function in US Children Awaiting Heart Transplant Maeda, K., Reinhartz, O., Zhang, Y., Chen, S., Murray, J., Gowen, M. M., McElhinney, D., Rosenthal, D. N., Dykes, J., Almond, C. ELSEVIER SCIENCE INC. 2017: S279–S280

  View details for Web of Science ID 000398839801003

• Development and validation of a major adverse transplant event (MATE) score to predict late graft loss in pediatric heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Almond, C. S., Hoen, H., Rossano, J. W., Castleberry, C., Auerbach, S. R., Yang, L., Lal, A. K., Everitt, M. D., Fenton, M., Hollander, S. A., Pahl, E., Pruitt, E., Rosenthal, D. N., McElhinney, D. B., Daly, K. P., Desai, M. 2017

  Abstract

  There is inadequate power to perform a valid clinical trial in pediatric heart transplantation (HT) using a conventional end-point, because the disease is rare and hard end-points, such as death or graft loss, are infrequent. We sought to develop and validate a surrogate end-point involving the cumulative burden of post-transplant complications to predict death/graft loss to power a randomized clinical trial of maintenance immunosuppression in pediatric HT.Pediatric Heart Transplant Study (PHTS) data were used to identify all children who underwent an isolated orthotopic HT between 2005 and 2014 who survived to 6 months post-HT. A time-varying Cox model was used to develop and evaluate a surrogate end-point comprised of 6 major adverse transplant events (MATEs) (acute cellular rejection [ACR], antibody-mediated rejection [AMR], infection, cardiac allograft vasculopathy [CAV], post-transplant lymphoproliferative disease [PTLD] and chronic kidney disease [CKD]) occurring between 6 and 36 months, where individual events were defined according to international guidelines. Two thirds of the study cohort was used for score development, and one third of the cohort was used to test the score.Among 2,118 children, 6.4% underwent graft loss between 6 and 36 months post-HT, whereas 39% developed CKD, 34% ACR, 34% infection, 9% AMR, 4% CAV and 2% PTLD. The best predictive score involved a simple MATE score sum, yielding a concordance probability estimate (CPE) statistic of 0.74. Whereas the power to detect non-inferiority (NI), assuming the NI hazard ratio of 1.45 in graft survival was 10% (assuming 200 subjects and 6% graft loss rate), the power to detect NI assuming a 2-point non-inferiority margin was >85% using the MATE score.The MATE score reflects the cumulative burden of MATEs and has acceptable prediction characteristics for death/graft loss post-HT. The MATE score may be useful as a surrogate end-point to power a clinical trial in pediatric HT.

  View details for DOI 10.1016/j.healun.2017.03.013

  View details for PubMedID 28465118

• INSURANCE TYPE IS ASSOCIATED WITH LONGER HOSPITAL DURATION BUT SIMILAR MORTALITY AMONG CHILDREN UNDERGOING CONGENITAL HEART SURGERY Ryan, K., Algaze, C., Sakarovitch, C., Do, T., Yarlagadda, V., Fernandes, S., McElhinney, D., Roth, S., Krawczeski, C., Shin, A. ELSEVIER SCIENCE INC. 2017: 569

  View details for Web of Science ID 000397342301091

• A multicenter study of the impella device for mechanical support of the systemic circulation in pediatric and adolescent patients. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Dimas, V. V., Morray, B. H., Kim, D. W., Almond, C. S., Shahanavaz, S., Tume, S. C., Peng, L. F., McElhinney, D. B., Justino, H. 2017

  Abstract

  The objective was to review the use of Impella devices (Abiomed Inc, Danvers, MA) for temporary circulatory support in pediatric and adolescent patients (age ≤ 21 yrs).Options for minimally invasive circulatory support in children are limited, and published data are confined to case reports and small case series.This was a retrospective, multicenter review of Impella implants in pediatric and adolescent patients from 2009-15, using standardized data collection and INTERMACS definitions.A total of 39 implants were performed in 38 patients from 16 centers. Median age and weight were 16 yrs (4-21 yrs) and 62 kg (15-134 kg). The primary indication for implant was cardiogenic shock in 28 patients (72%). Cardiac allograft rejection, myocarditis, or cardiomyopathy were the underlying diagnosis in 23 patients (59%); 11 patients had congenital heart disease. The median duration of support was 45 hr (1-1224 hr). Indications for explant included ventricular recovery in 16 patients, transition to another device in 12, death in 5, and transplant in 1. Survival was 85% at 7 days and 68% at 30 days. Major adverse events occurred in 8 patients: hemolysis in 3, bleeding in 2, stroke in 1 (unclear if related to Impella), sepsis in 1, and critical leg ischemia in 1. An increase in aortic regurgitation was noted in three patients, with no evidence of valve injury.Temporary circulatory support with Impella devices is feasible in pediatric and adolescent patients, with acceptable risk profiles. More experience and follow up is needed to improve technical performance and patient selection. © 2017 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.26973

  View details for PubMedID 28295963

• Functional status of United States children supported with a left ventricular assist device at heart transplantation. journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Bulic, A., Maeda, K., Zhang, Y., Chen, S., McElhinney, D. B., Dykes, J. C., Hollander, A. M., Hollander, S. A., Murray, J., Reinhartz, O., Gowan, M. A., Rosenthal, D. N., Almond, C. S. 2017

  Abstract

  As survival with pediatric left ventricular assist devices (LVADs) has improved, decisions regarding the optimal support strategy may depend more on quality of life and functional status (FS) rather than mortality alone. Limited data are available regarding the FS of children supported with LVADs. We sought to compare the FS of children supported with LVADs vs vasoactive infusions to inform decision making around support strategies.Organ Procurement and Transplant Network data were used to identify all United States children aged between 1 and 21 years at heart transplant (HT) between 2006 and 2015 for dilated cardiomyopathy and supported with an LVAD or vasoactive infusions alone at HT. FS was measured using the 10-point Karnofsky and Lansky scale.Of 701 children who met the inclusion criteria, 430 (61%) were supported with vasoactive infusions, and 271 (39%) were supported with an LVAD at HT. Children in the LVAD group had higher median FS scores at HT than children in the vasoactive infusion group (6 vs 5, p < 0.001) but lower FS scores at listing (4 vs 6, p < 0.001). The effect persisted regardless of patient location at HT (home, hospital, intensive care) or device type. Discharge by HT occurred in 46% of children in the LVAD group compared with 26% of children in the vasoactive infusion cohort (p = 0.001). Stroke was reported at HT in 3% of children in the LVAD cohort and in 1% in the vasoactive infusion cohort (p = 0.04).Among children with dilated cardiomyopathy undergoing HT, children supported with LVADs at HT have higher FS than children supported with vasoactive infusions at HT, regardless of device type or hospitalization status. Children supported with LVADs at HT were more likely to be discharged from the hospital but had a higher prevalence of stroke at HT.

  View details for DOI 10.1016/j.healun.2017.02.024

  View details for PubMedID 28363739

• Transcatheter valve implantation for right atrium-to-right ventricle conduit obstruction or regurgitation after modified Björk-fontan procedure. Catheterization and cardiovascular interventions Shah, A. H., Horlick, E. M., Eicken, A., Asnes, J. D., Bocks, M. L., Boudjemline, Y., Cabalka, A. K., Fagan, T. E., Schubert, S., Mahadevan, V. S., Dvir, D., Osten, M., McElhinney, D. B. 2017; 89 (2): 298-305

  Abstract

  The most common reason for reintervention after a Björk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway.Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA-RV conduit or valve.All patients had successful and uncomplicated implantation of a Melody (n = 15) or Sapien 3 (n = 1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed.Percutaneous transcatheter valve deployment to treat a dysfunctional RA-RV connection after a Björk modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results.© 2016 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.26648

  View details for PubMedID 27465501

• Reflection and Rationalization: Making Sense of the Literature on Endocarditis After Transcatheter Pulmonary Valve Replacement. Circulation. Cardiovascular interventions McElhinney, D. B. 2017; 10 (2)

  View details for DOI 10.1161/CIRCINTERVENTIONS.117.004983

  View details for PubMedID 28153843

• Rehospitalization after pediatric heart transplantation: Incidence, indications, and outcomes. Pediatric transplantation Hollander, S. A., McElhinney, D. B., Almond, C. S., McDonald, N., Chen, S., Kaufman, B. D., Bernstein, D., Rosenthal, D. N. 2017; 21 (1)

  Abstract

  We report the patterns of rehospitalization after pediatric heart transplant (Htx) at a single center. Retrospective review of 107 consecutive pediatric Htx recipients between January 22, 2007, and August 28, 2014, who survived their initial transplant hospitalization. The frequency, duration, and indications for all hospitalizations between transplant hospitalization discharge and September 30, 2015, were analyzed. A total of 444 hospitalization episodes occurred in 90 of 107 (84%) patients. The median time to first rehospitalization was 59.5 (range 1-1526) days, and the median length of stay was 2.5 (range 0-81) days. There were an average of two hospitalizations per patient in the first year following transplant hospitalization, declining to about 0.8 per patient per year starting at 3 years post-transplant. Admissions for viral infections were most common, occurring in 93 of 386 (24%), followed by rule out sepsis in 61 of 386 (16%). Admissions for suspected or confirmed rejection were less frequent, accounting for 41 of 386 (11%) and 31 of 386 (8%) of all admissions, respectively. Survival to discharge after rehospitalization was 97%. Hospitalization is common after pediatric Htx, particularly in the first post-transplant year, with the most frequent indications for hospitalization being viral illness and rule out sepsis. After the first post-transplant year, the risk for readmission falls significantly but remains constant for several years.

  View details for DOI 10.1111/petr.12857

  View details for PubMedID 27891727

• Circulatory support using the impella device in fontan patients with systemic ventricular dysfunction: A multicenter experience. Catheterization and cardiovascular interventions Morray, B. H., Dimas, V. V., Lim, S., Balzer, D. T., Parekh, D. R., Van Mieghem, N. M., Ewert, P., Kim, D. W., Justino, H., McElhinney, D. B., Jones, T. K. 2017

  Abstract

  There are limited mechanical circulatory support options for patients with single ventricle (SV) anatomy. This is a multicenter, retrospective study of the Impella pump to support the systemic ventricle in a cohort of SV patients with Fontan circulation.Patients with SV anatomy supported with an Impella device from 2012 to 2015 were included. Demographic information, indication for support, adverse events and short-term outcome data were collected.Ten patients were included. The median age and weight at implant was 26 years (4-38 years) and 64 kg (15-102 kg). Indications for support were systemic ventricular failure with cardiogenic shock (n = 8) or high-risk electrophysiology (EP) procedures (n = 2). The median duration of support was 49 hr (2.7-264 hr). Support was discontinued for ventricular recovery in five patients, transition to another device in two patients, completion of EP procedure in two patients and death in one patient. Survival to hospital discharge was 80%. Adverse events occurred in 4 patients. There were two cases of hemolysis, one case of increasing aortic valve insufficiency with implant and one asymptomatic access site thrombus. There were no bleeding or thromboembolic events.Impella devices can provide temporary support for the systemic ventricle in SV patients as a bridge to recovery or additional device. Procedural survival and adverse event profiles are favorable. © 2017 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.26885

  View details for PubMedID 28112463

• Transcatheter Valve-in-Ring Implantation for the Treatment of Residual or Recurrent Tricuspid Valve Dysfunction After Prior Surgical Repair JACC-CARDIOVASCULAR INTERVENTIONS Aboulhosn, J., Cabalka, A. K., Levi, D. S., Himbert, D., Testa, L., Latib, A., Makkar, R. R., Boudjemline, Y., Kim, D. W., Kefer, J., Bleiziffer, S., Kerst, G., Dvir, D., McElhinney, D. B. 2017; 10 (1): 53-63

  Abstract

  This study sought to describe the results of transcatheter tricuspid valve-in-ring (TVIR) implantation for treatment of tricuspid regurgitation (TR).Off-label use of transcatheter valves within surgically placed tricuspid annuloplasty prostheses has only been described in small reports. An international multicenter registry was developed to collect data on TVIR implantation.Data were collected from 13 sites on 22 patients (5 to 69 years of age) with TR who underwent catheterization with the intent to perform TVIR implantation.TVIR implantation was performed in 20 patients (91%). Most patients were severely impaired (86% in New York Heart Association functional class III or IV); TR was severe in 86%. A Sapien valve (Edwards Lifesciences, Irvine, California) was implanted in 17 patients and a Melody valve (Medtronic, Minneapolis, Minnesota) in 3. There were no procedural deaths. There was 1 valve embolization requiring retrieval and placement of second TVIR implant and 1 valve malposition with severe paravalvular regurgitation requiring a second TVIR implantation. Over a median follow-up of 12 months, 1 patient died and 2 underwent repeat TVIR implantation, 1 of whom subsequently underwent surgical valve replacement. Significant paravalvular leak (PVL) was treated at the time of TVIR implantation in 4 patients: 3 underwent device occlusion and 1 received a second TVIR implant. On follow-up echocardiography, 15 patients had PVL (75%), the majority of which (n = 10) were trivial or mild and did not require treatment. PVL intervention was performed in 3 patients during follow-up. Functional capacity improved in most patients (70%).TVIR implantation using commercially available transcatheter prostheses is technically feasible and clinically effective in reducing TR. Paravalvular regurgitation is common and may necessitate further interventions.

  View details for DOI 10.1016/j.jcin.2016.10.036

  View details for Web of Science ID 000392624100010

  View details for PubMedID 28057286

• Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118

  Abstract

  The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

  View details for DOI 10.1016/j.amjcard.2016.09.023

  View details for PubMedID 28247847

• Estimating One-Year Risk of Incident Chronic Kidney Disease: Retrospective Development and Validation Study Using Electronic Medical Record Data From the State of Maine. JMIR medical informatics Hao, S. n., Fu, T. n., Wu, Q. n., Jin, B. n., Zhu, C. n., Hu, Z. n., Guo, Y. n., Zhang, Y. n., Yu, Y. n., Fouts, T. n., Ng, P. n., Culver, D. S., Alfreds, S. T., Stearns, F. n., Sylvester, K. G., Widen, E. n., McElhinney, D. B., Ling, X. B. 2017; 5 (3): e21

  Abstract

  Chronic kidney disease (CKD) is a major public health concern in the United States with high prevalence, growing incidence, and serious adverse outcomes.We aimed to develop and validate a model to identify patients at risk of receiving a new diagnosis of CKD (incident CKD) during the next 1 year in a general population.The study population consisted of patients who had visited any care facility in the Maine Health Information Exchange network any time between January 1, 2013, and December 31, 2015, and had no history of CKD diagnosis. Two retrospective cohorts of electronic medical records (EMRs) were constructed for model derivation (N=1,310,363) and validation (N=1,430,772). The model was derived using a gradient tree-based boost algorithm to assign a score to each individual that measured the probability of receiving a new diagnosis of CKD from January 1, 2014, to December 31, 2014, based on the preceding 1-year clinical profile. A feature selection process was conducted to reduce the dimension of the data from 14,680 EMR features to 146 as predictors in the final model. Relative risk was calculated by the model to gauge the risk ratio of the individual to population mean of receiving a CKD diagnosis in next 1 year. The model was tested on the validation cohort to predict risk of CKD diagnosis in the period from January 1, 2015, to December 31, 2015, using the preceding 1-year clinical profile.The final model had a c-statistic of 0.871 in the validation cohort. It stratified patients into low-risk (score 0-0.005), intermediate-risk (score 0.005-0.05), and high-risk (score ≥ 0.05) levels. The incidence of CKD in the high-risk patient group was 7.94%, 13.7 times higher than the incidence in the overall cohort (0.58%). Survival analysis showed that patients in the 3 risk categories had significantly different CKD outcomes as a function of time (P<.001), indicating an effective classification of patients by the model.We developed and validated a model that is able to identify patients at high risk of having CKD in the next 1 year by statistically learning from the EMR-based clinical history in the preceding 1 year. Identification of these patients indicates care opportunities such as monitoring and adopting intervention plans that may benefit the quality of care and outcomes in the long term.

  View details for PubMedID 28747298

• Impact of the 18th birthday on waitlist outcomes among young adults listed for heart transplant: A regression discontinuity analysis. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Peng, D. M., Qu, Q. n., McDonald, N. n., Hollander, S. A., Bernstein, D. n., Maeda, K. n., Kaufman, B. D., Rosenthal, D. N., McElhinney, D. B., Almond, C. S. 2017; 36 (11): 1185–91

  Abstract

  Patients listed for heart transplant after their 18th birthday purportedly wait longer to receive a donor heart compared with patients listed before their 18th birthday. It is unclear whether there is an actual difference in wait times and whether any difference in wait time is associated with lower likelihood of transplant and/or higher risk of mortality.Organ procurement and transplant network data were used to identify all patients listed for heart transplant between 2006 and 2014 within a 1-year period before and after their 18th birthday. The primary study end-point was the waiting time to receive a donor heart. Secondary end-points included the probability of transplant and waitlist mortality. Regression discontinuity analysis was used to analyze the effect of age on either side of the sharp cut-off value of age 18 years (6,574 days of life), when allocation of donor hearts transitions from the pediatric to adult allocation system.A total of 360 patients met the study inclusion criteria, including 207 (57.5%) listed during the 12-month period before their 18th birthday under the pediatric allocation system, and 153 (42.5%) listed during the 12 months after their 18th birthday under the adult allocation system. The pediatric cohort was more likely to be listed Status 1A. Otherwise, the 2 groups shared similar baseline characteristics. Overall, patients listed after their 18th birthday waited 8.5 months longer to receive a transplant than adolescents listed before their 18th birthday (p = 0.01) and had a 47% lower probability of receiving a transplant (p = 0.001), but there was no difference in waitlist mortality (p = 0.37).Patients listed for heart transplant shortly after their 18th birthday have significantly longer wait-times compared with patients listed shortly before their 18th birthday and a lower probability of transplant, but no significant difference in waitlist mortality. For medically fragile adolescents at high risk of death, birth date may be a relevant factor in the timing of heart transplant listing.

  View details for PubMedID 28712678

• Haemodynamic profiles of children with end-stage heart failure. European heart journal Chen, S. n., Dykes, J. C., McElhinney, D. B., Gajarski, R. J., Shin, A. Y., Hollander, S. A., Everitt, M. E., Price, J. F., Thiagarajan, R. R., Kindel, S. J., Rossano, J. W., Kaufman, B. D., May, L. J., Pruitt, E. n., Rosenthal, D. N., Almond, C. S. 2017; 38 (38): 2900–2909

  Abstract

  To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation.Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist. Among 1059 children analysed, median age was 6.9 years and 46% had dilated cardiomyopathy. Overall, 58% had congestion (PCWP >15 mmHg), 28% had severe congestion (PCWP >22 mmHg), and 22% low cardiac output (CI < 2.2 L/min/m2). Twenty-one per cent met the primary outcome of death (9%) or clinical deterioration (12%). In multivariable analysis, worse NYHA/Ross classification was associated with increased PCWP [odds ratio (OR) 1.03, 95% confidence interval (95% CI) 1.01-1.07, P = 0.01], renal dysfunction with increased RAP (OR 1.04, 95% CI 1.01-1.08, P = 0.007), and hepatic dysfunction with both increased PCWP (OR 1.03, 95% CI 1.01-1.06, P < 0.001) and increased RAP (OR 1.09, 95% CI 1.06-1.12, P < 0.001). There were no associations with low output. Death or clinical deterioration was associated with severe congestion (OR 1.6, 95% CI 1.2-2.2, P = 0.002), but not with CI alone. However, children with both low output and severe congestion were at highest risk (OR 1.9, 95% CI 1.1-3.5, P = 0.03).Congestion is more common than low cardiac output in children with end-stage heart failure and correlates with NYHA/Ross classification and end-organ dysfunction. Children with both congestion and low output have the highest risk of death or clinical deterioration.

  View details for PubMedID 29019615

• Temporary Circulatory Support in U.S. Children Awaiting Heart Transplantation. Journal of the American College of Cardiology Yarlagadda, V. V., Maeda, K. n., Zhang, Y. n., Chen, S. n., Dykes, J. C., Gowen, M. A., Shuttleworth, P. n., Murray, J. M., Shin, A. Y., Reinhartz, O. n., Rosenthal, D. N., McElhinney, D. B., Almond, C. S. 2017; 70 (18): 2250–60

  Abstract

  Extracorporeal membrane oxygenation (ECMO) has long served as the standard of care for short-term mechanical circulatory support in pediatrics. It is unknown whether newer-generation temporary circulatory support (TCS) devices afford children a meaningful survival advantage over ECMO.This study sought to determine whether bridge-to-heart transplant survival with a TCS device is superior to ECMO after adjusting for patient differences.All children ≤21 years of age listed for heart transplant from 2011 to 2015 who received a TCS device or ECMO as a bridge to transplant were identified using Organ Procurement and Transplantation Network data. Children supported with a TCS device were compared with a propensity score (PS)-matched cohort of children supported with ECMO as a bridge to transplant. The primary endpoint was Kaplan-Meier survival to transplant.The number of TCS devices implanted in children increased from ≤3 per year before 2011 to 50 in 2015. Overall, 93 patients implanted with TCS devices were included for analysis (59% left ventricular assist devices, 23% right ventricular assist devices, 18% biventricular assist devices). The most commonly used device was the CentriMag-PediMag system (65%), followed by TandemHeart (18%), Rotaflow (6%), and Impella (5%). Among 164 PS-matched patients, support duration was longer for the TCS cohort (median 19 days vs. 6 days; p < 0.001), and was longest for the CentriMag-PediMag (24 days vs. 6 days; p < 0.001) with 27% supported for >60 days. Compared with the ECMO cohort, the PS-matched TCS cohort had longer survival to transplant (hazard ratio: 0.49; 95% confidence interval: 0.30 to 0.79) and longer overall survival (hazard ratio: 0.61; 95% confidence interval: 0.39 to 0.96), with 90-day mortality before transplant that was modestly reduced (from 45% with ECMO to 39% with TCS).The use of TCS devices in children as a bridge to transplant has risen rapidly in recent years, led by the growth of magnetically levitated centrifugal flow pumps. Compared with conventional ECMO, TCS durations are longer, and more importantly, patient survival is superior.

  View details for PubMedID 29073953

• Impact of a modified anti-thrombotic guideline on stroke in children supported with a pediatric ventricular assist device. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Rosenthal, D. N., Lancaster, C. A., McElhinney, D. B., Chen, S. n., Stein, M. n., Lin, A. n., Doan, L. n., Murray, J. M., Gowan, M. A., Maeda, K. n., Reinhartz, O. n., Almond, C. S. 2017

  Abstract

  Stroke is the most feared complication associated with the Berlin Heart EXCOR pediatric ventricular assist device (VAD), the most commonly used VAD in children, and affects 1 in 3 children. We sought to determine whether a modified anti-thrombotic guideline, involving more intense platelet inhibition and less reliance on platelet function testing, is associated with a lower incidence of stroke.All children supported with the EXCOR at Stanford from 2009 to 2014 were divided into 2 cohorts based on the primary anti-thrombotic guideline used to prevent pump thrombosis: (1) the Edmonton Anti-thrombotic Guideline (EG) cohort, which included children implanted before September 2012 when dual anti-platelet therapy was used with doses titrated to Thromboelastrography/PlateletMapping (TEG/PM); and (2) the Stanford Modified Anti-thrombotic Guideline (SG) cohort, which included children implanted on or after September 2012 when triple anti-platelet therapy was used routinely and where doses were uptitrated to high, weight-based dosing targets, with low-dose steroids administered as needed for inflammation.At baseline, the EG (N = 16) and SG (N = 11) cohorts were similar. The incidence rate of stroke in the SG cohort was 84% lower than in the EG cohort (0.8 vs 4.9 events per 1,000 days of support, p = 0.031), and 86% lower than in the previous Investigational Device Exemption trial (p = 0.006). The bleeding rate was also lower in the SG cohort (p = 0.015). Target doses of aspirin, clopidogrel and dipyridamole were higher (all p < 0.003), with less dosing variability in the SG cohort than in the EG cohort. There was no difference in adenosine diphosphate inhibition by TEG/PM, but arachidonic acid inhibition was higher in the SG cohort (median 75% vs 39%, p = 0.008).Stroke was significantly less common in pediatric patients supported with the Berlin Heart EXCOR VAD using a triple anti-platelet regimen uptitrated to high, weight-based dosing targets as compared with the dual anti-platelet regimen titrated to PM, and without a higher risk of bleeding. Larger studies are needed to confirm these findings.

  View details for PubMedID 28606584

• Defining and characterizing the critical transition state prior to the type 2 diabetes disease. PloS one Jin, B. n., Liu, R. n., Hao, S. n., Li, Z. n., Zhu, C. n., Zhou, X. n., Chen, P. n., Fu, T. n., Hu, Z. n., Wu, Q. n., Liu, W. n., Liu, D. n., Yu, Y. n., Zhang, Y. n., McElhinney, D. B., Li, Y. M., Culver, D. S., Alfreds, S. T., Stearns, F. n., Sylvester, K. G., Widen, E. n., Ling, X. B. 2017; 12 (7): e0180937

  Abstract

  Type 2 diabetes mellitus (T2DM), with increased risk of serious long-term complications, currently represents 8.3% of the adult population. We hypothesized that a critical transition state prior to the new onset T2DM can be revealed through the longitudinal electronic medical record (EMR) analysis.We applied the transition-based network entropy methodology which previously identified a dynamic driver network (DDN) underlying the critical T2DM transition at the tissue molecular biological level. To profile pre-disease phenotypical changes that indicated a critical transition state, a cohort of 7,334 patients was assembled from the Maine State Health Information Exchange (HIE). These patients all had their first confirmative diagnosis of T2DM between January 1, 2013 and June 30, 2013. The cohort's EMRs from the 24 months preceding their date of first T2DM diagnosis were extracted.Analysis of these patients' pre-disease clinical history identified a dynamic driver network (DDN) and an associated critical transition state six months prior to their first confirmative T2DM state.This 6-month window before the disease state provides an early warning of the impending T2DM, warranting an opportunity to apply proactive interventions to prevent or delay the new onset of T2DM.

  View details for PubMedID 28686739

• Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience JOURNAL OF PEDIATRICS Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Tierney, E. S. 2017; 180: 87-?

  Abstract

  To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

  View details for DOI 10.1016/j.jpeds.2016.09.061

  View details for Web of Science ID 000390028100018

• Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions? Pediatric cardiology Bhatla, P., Tretter, J. T., Ludomirsky, A., Argilla, M., Latson, L. A., Chakravarti, S., Barker, P. C., Yoo, S., McElhinney, D. B., Wake, N., Mosca, R. S. 2017; 38 (1): 103-114

  Abstract

  Rapid prototyping facilitates comprehension of complex cardiac anatomy. However, determining when this additional information proves instrumental in patient management remains a challenge. We describe our experience with patient-specific anatomic models created using rapid prototyping from various imaging modalities, suggesting their utility in surgical and interventional planning in congenital heart disease (CHD). Virtual and physical 3-dimensional (3D) models were generated from CT or MRI data, using commercially available software for patients with complex muscular ventricular septal defects (CMVSD) and double-outlet right ventricle (DORV). Six patients with complex anatomy and uncertainty of the optimal management strategy were included in this study. The models were subsequently used to guide management decisions, and the outcomes reviewed. 3D models clearly demonstrated the complex intra-cardiac anatomy in all six patients and were utilized to guide management decisions. In the three patients with CMVSD, one underwent successful endovascular device closure following a prior failed attempt at transcatheter closure, and the other two underwent successful primary surgical closure with the aid of 3D models. In all three cases of DORV, the models provided better anatomic delineation and additional information that altered or confirmed the surgical plan. Patient-specific 3D heart models show promise in accurately defining intra-cardiac anatomy in CHD, specifically CMVSD and DORV. We believe these models improve understanding of the complex anatomical spatial relationships in these defects and provide additional insight for pre/intra-interventional management and surgical planning.

  View details for DOI 10.1007/s00246-016-1489-1

  View details for PubMedID 27837304

• Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia AMERICAN JOURNAL OF CARDIOLOGY Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2017; 119 (1): 106-111

  Abstract

  In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

  View details for DOI 10.1016/j.amjcard.2016.09.022

  View details for Web of Science ID 000391246900017

• Unique Molecular Patterns Uncovered in Kawasaki Disease Patients with Elevated Serum Gamma Glutamyl Transferase Levels: Implications for Intravenous Immunoglobulin Responsiveness PLOS ONE Wang, Y., Li, Z., Hu, G., Hao, S., Deng, X., Huang, M., Ren, M., Jiang, X., Kanegaye, J. T., Ha, K., Lee, J., Li, X., Jiang, X., Yu, Y., Tremoulet, A. H., Burns, J. C., Whitin, J. C., Shin, A. Y., Sylvester, K. G., McElhinney, D. B., Cohen, H. J., Ling, X. B. 2016; 11 (12)

  Abstract

  Resistance to intravenous immunoglobulin (IVIG) occurs in 10-20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels.We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I). Gene expression data from 130 children with acute KD (Cohort II) were analyzed using the R square statistic and false discovery analysis to identify genes that were differentially represented in patients with elevated GGT levels with regard to IVIG responsiveness. Two additional KD cohorts (Cohort III and IV) were used to test the hypothesis that sialylation and GGT may be involved in IVIG resistance through neutrophil apoptosis.Thirty-six genes were identified that significantly explained the variations of both GGT levels and IVIG responsiveness in KD patients. After Bonferroni correction, significant associations with IVIG resistance persisted for 12 out of 36 genes among patients with elevated GGT levels and none among patients with normal GGT levels. With the discovery of ST6GALNAC3, a sialyltransferase, as the most differentially expressed gene, we hypothesized that sialylation and GGT are involved in IVIG resistance through neutrophil apoptosis. We then confirmed that in Cohort III and IV there was significantly less reduction in neutrophil count in IVIG non-responders.Gene expression analyses combining molecular and clinical datasets support the hypotheses that: (1) neutrophil apoptosis induced by IVIG may be a mechanism of action of IVIG in KD; (2) changes in sialylation and GGT level in KD patients may contribute synergistically to IVIG resistance through blocking IVIG-induced neutrophil apoptosis. These findings have implications for understanding the mechanism of action in IVIG resistance, and possibly for development of novel therapeutics.

  View details for DOI 10.1371/journal.pone.0167434

  View details for Web of Science ID 000392853100008

  View details for PubMedID 28002448

  View details for PubMedCentralID PMC5176264

• Web-based Real-Time Case Finding for the Population Health Management of Patients With Diabetes Mellitus: A Prospective Validation of the Natural Language Processing-Based Algorithm With Statewide Electronic Medical Records. JMIR medical informatics Zheng, L., Wang, Y., Hao, S., Shin, A. Y., Jin, B., Ngo, A. D., Jackson-Browne, M. S., Feller, D. J., Fu, T., Zhang, K., Zhou, X., Zhu, C., Dai, D., Yu, Y., Zheng, G., Li, Y., McElhinney, D. B., Culver, D. S., Alfreds, S. T., Stearns, F., Sylvester, K. G., Widen, E., Ling, X. B. 2016; 4 (4)

  Abstract

  Diabetes case finding based on structured medical records does not fully identify diabetic patients whose medical histories related to diabetes are available in the form of free text. Manual chart reviews have been used but involve high labor costs and long latency.This study developed and tested a Web-based diabetes case finding algorithm using both structured and unstructured electronic medical records (EMRs).This study was based on the health information exchange (HIE) EMR database that covers almost all health facilities in the state of Maine, United States. Using narrative clinical notes, a Web-based natural language processing (NLP) case finding algorithm was retrospectively (July 1, 2012, to June 30, 2013) developed with a random subset of HIE-associated facilities, which was then blind tested with the remaining facilities. The NLP-based algorithm was subsequently integrated into the HIE database and validated prospectively (July 1, 2013, to June 30, 2014).Of the 935,891 patients in the prospective cohort, 64,168 diabetes cases were identified using diagnosis codes alone. Our NLP-based case finding algorithm prospectively found an additional 5756 uncodified cases (5756/64,168, 8.97% increase) with a positive predictive value of .90. Of the 21,720 diabetic patients identified by both methods, 6616 patients (6616/21,720, 30.46%) were identified by the NLP-based algorithm before a diabetes diagnosis was noted in the structured EMR (mean time difference = 48 days).The online NLP algorithm was effective in identifying uncodified diabetes cases in real time, leading to a significant improvement in diabetes case finding. The successful integration of the NLP-based case finding algorithm into the Maine HIE database indicates a strong potential for application of this novel method to achieve a more complete ascertainment of diagnoses of diabetes mellitus.

  View details for PubMedID 27836816

• Subacute left ventricular outflow tract obstruction after transapical closure of a mitral paravalvular leak in the region of the aortomitral curtain ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Chikkabyrappa, S., McElhinney, D. B., Saric, M. 2016; 33 (11): 1771-1776

  Abstract

  We report a rare case of progressive left ventricular outflow tract (LVOT) obstruction after percutaneous device closure of a mechanical prosthetic mitral valve (MV) paravalvular leak (PVL) in the region of aortomitral curtain in a patient who also had small mechanical aortic valve prosthesis with patient-prosthesis mismatch.

  View details for DOI 10.1111/echo.13357

  View details for Web of Science ID 000388309500021

  View details for PubMedID 27576330

• Hybrid transcatheter pulmonary valve replacement: Moving into the mainstream? CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2016; 88 (5): 811–13

  View details for PubMedID 27865074

• Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience. journal of pediatrics Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Selamet Tierney, E. S. 2016

  Abstract

  To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

  View details for DOI 10.1016/j.jpeds.2016.09.061

  View details for PubMedID 28029346

• Defining and refining indications for transcatheter pulmonary valve replacement in patients with repaired tetralogy of Fallot: Contributions from anatomical and functional imaging. International journal of cardiology Tretter, J. T., Friedberg, M. K., Wald, R. M., McElhinney, D. B. 2016; 221: 916-925

  Abstract

  Transcatheter pulmonary valve replacement (TPVR) is an important treatment option in repaired tetralogy of Fallot (TOF) and right ventricular outflow tract (RVOT) dysfunction. Indications for timing of TPVR are extrapolated from surgical pulmonary valve replacement guidelines, which are themselves controversial as published evidence is scarce and expert opinion therefore prevails. We review current indications for PVR following TOF repair, focusing on those for TPVR specifically, and discuss anatomical and functional considerations as these pertain to determination of candidacy for TPVR. Hemodynamic assessment surrounding PVR has focused on assessment of the right ventricle (RV) size and systolic function, with the goal of intervening in the asymptomatic patient prior to the development of irreversible RV deterioration and right heart failure. The impact of abnormal RV mechanics on the LV has been appreciated, with the assessment of LV function assuming higher priority in decision-making regarding possible PVR. In addition to the standard volumetric assessment, evaluation with indices of myocardial wall strain, tissue velocities, diastology, and ventricular response to exercise is emerging as tools with potential to further refine timing of PVR. We conclude that, at present, current evidence, although limited, supports a more aggressive approach in those who meet inclusion for TPVR in patients with repaired TOF and RVOT dysfunction guided by the discussed hemodynamic assessment, however, more importantly this review should lay the framework for future investigations regarding hemodynamic assessment of this population.

  View details for DOI 10.1016/j.ijcard.2016.07.120

  View details for PubMedID 27441469

• Transcatheter Pulmonary Valve Replacement Reduces Tricuspid Regurgitation in Patients With Right Ventricular Volume/Pressure Overload. Journal of the American College of Cardiology Jones, T. K., Rome, J. J., Armstrong, A. K., Berger, F., Hellenbrand, W. E., Cabalka, A. K., Benson, L. N., Balzer, D. T., Cheatham, J. P., Eicken, A., McElhinney, D. B. 2016; 68 (14): 1525-1535

  Abstract

  Tricuspid regurgitation (TR) is a common and important comorbidity in patients with postoperative right ventricular outflow tract (RVOT) obstruction or pulmonary regurgitation (PR). Transcatheter pulmonary valve replacement (TPVR) has become a useful tool in the management of postoperative RVOT obstruction and PR, but it is unknown whether relief of the right ventricular volume and/or pressure overload by TPVR will have a beneficial effect on TR, as is often seen with surgical pulmonary valve replacement.This study sought to assess the prevalence of and factors associated with significant TR in patients undergoing TPVR for RVOT obstruction or PR.Data were combined from 3 prospective multicenter trials of patients referred for TPVR. Follow-up data through 5 years post-implantation were analyzed.Of 300 patients studied, 77 (25.6%) had moderate or severe TR at baseline. After TPVR, TR severity was improved in 65% of those patients, and more than one-half had mild TR or less TR at discharge. Of 13 patients with severe TR pre-implantation, only 1 had severe TR at 1-year follow-up and beyond. Moderate or severe baseline TR was associated with shorter freedom from RVOT reintervention after TPVR.In this prospective multicenter study of post-operative patients with RVOT obstruction and/or PR, TR was common. In patients with significant baseline TR, TPVR resulted in clinically relevant acute reductions in TR that persisted over at least 5 years of follow-up. These observations support the application of TPVR therapy in patients with RVOT obstruction or PR who are anatomically suitable, even in the setting of significant concomitant TR.

  View details for DOI 10.1016/j.jacc.2016.07.734

  View details for PubMedID 27687194

• Assessment of Progressive Pathophysiology After Early Prenatal Diagnosis of the Ebstein Anomaly or Tricuspid Valve Dysplasia. American journal of cardiology Selamet Tierney, E. S., McElhinney, D. B., Freud, L. R., Tworetzky, W., Cuneo, B. F., Escobar-Diaz, M. C., Ikemba, C., Kalish, B. T., Komarlu, R., Levasseur, S. M., Puchalski, M. D., Satou, G. M., Silverman, N. H., Moon-Grady, A. J. 2016

  Abstract

  In fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD), poor hemodynamic status is associated with worse neonatal outcome. It is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in the third trimester. We evaluated if echocardiographic indexes in EA/TVD fetuses presenting <24 weeks of gestation are reliable indicators of physiologic status later in pregnancy. This multicenter, retrospective study included 51 fetuses presenting at <24 weeks of gestation with EA/TVD and serial fetal echocardiograms ≥4 weeks apart. We designated the following as markers of poor outcome: absence of anterograde flow across the pulmonary valve, pulmonary valve regurgitation, cardiothoracic area ratio >0.48, left ventricular (LV) dysfunction, or tricuspid valve (TV) annulus Z-score >5.6. Median gestational age at diagnosis was 21 weeks (range, 18 to 24). Eighteen fetuses (35%) had no markers for poor hemodynamic status initially, whereas only 7 of these continued to have no markers of poor outcome in the third trimester. Nine of 27 fetuses (33%) with anterograde pulmonary blood flow on the first echocardiogram developed pulmonary atresia; 7 of 39 (18%) developed new pulmonary valve regurgitation. LV dysfunction was present in 2 (4%) patients at <24 weeks but in 14 (37%) later (p <0.001). The TV annulus Z-score and cardiothoracic area both increased from diagnosis to follow-up. In conclusion, progressive hemodynamic compromise was common in this cohort. Our study highlights that care must be taken in counseling before 24 weeks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.

  View details for DOI 10.1016/j.amjcard.2016.09.022

  View details for PubMedID 27793395

• Progressive intermediate-term improvement in ventricular and atrioventricular interaction after transcatheter pulmonary valve replacement in patients with right ventricular outflow tract obstruction. American heart journal Lunze, F. I., Hasan, B. S., Gauvreau, K., Brown, D. W., Colan, S. D., McElhinney, D. B. 2016; 179: 87-98

  Abstract

  Relief of postoperative right ventricular outflow tract (RVOT) obstruction with transcatheter pulmonary valve replacement (TPVR) results in functional improvement in the short term which we investigated at baseline (BL), early follow-up (FU), and midterm FU after TPVR.Echocardiography and cardiopulmonary exercise testing were performed at BL and at early (median 6 months) and midterm FU (median 2.5years) after TPVR.Patients with RVOT obstruction (n=22, median age 17years) were studied. The max RVOT Doppler gradient fell from BL to early FU (60±24 to 26±8mm Hg, P<.001). Left ventricular (LV) end-diastolic and stroke volume increased at early FU (both P<.001) without further change, whereas LV ejection fraction improved throughout FU (P<.001). LV end-systolic and diastolic eccentricity (leftward septal displacement) improved early (both P≤.003), and end-diastolic eccentricity improved further at midterm FU (P=.02). Furthermore, whereas mitral inflow A wave velocity increased (P=.003), the LV A' velocity declined early (P=.007) without further change at midterm. RV systolic and early diastolic function was impaired at BL. Whereas RV strain improved partially at early and midterm FU (P≤.02), RV E' velocity did not improve throughout FU. Mildly impaired LV strain at BL fully recovered by midterm FU (P≤.002). Peak oxygen uptake improved at early and midterm FU (all P≤.003).Patients with RVOT obstruction had biventricular systolic and diastolic dysfunction at BL. Relieving RVOT obstruction with TPVR reduced adverse ventricular and compensatory atrioventricular interaction, resulting in progressive biventricular functional improvement and remodeling at early and midterm FU.

  View details for DOI 10.1016/j.ahj.2016.05.011

  View details for PubMedID 27595683

• Sequential Percutaneous Closure of Mitral Prosthetic Paravalvular Leak and Complex Communicating Pseudoaneurysms of the Ascending Aorta and Subvalvar Left Ventricular Outflow Tract CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Tretter, J. T., Latson, L. A., McElhinney, D. B. 2016; 88 (1): 150-156

  Abstract

  Ascending aortic and subvalvar left ventricular outflow tract (LVOT) pseudoaneurysms are rare complications following aortic valve or root replacement surgery. Clinically important paravalvular leaks are rare complications following any valve replacement surgery. We report an unusual case of sequential percutaneous closure of mitral prosthetic paravalvular leak and complex communicating ascending aortic and subvalvar LVOT pseudoaneurysms, which demonstrates the importance of multimodal imaging assessment surrounding percutaneous closure. © 2015 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.26001

  View details for Web of Science ID 000379984000031

  View details for PubMedID 25964108

• Identifying Gaps in Technology for Congenital Interventions: Analysis of a Needs Survey from Congenital Interventional Cardiologists PEDIATRIC CARDIOLOGY Shibbani, K., Kenny, D., McElhinney, D., Hijazi, Z. M., Moran, T. 2016; 37 (5): 925-931

  Abstract

  We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41 % ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23 % ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27 % reporting that covered stents would have the greatest impact. Fifty percent of participants reported that they would like to see large covered stents available in their country. These data point toward a perceived need for the development/approval of bioresorbable stents for the pediatric age group, as well as the need to approve the use of large covered stents in the pediatric age group in the USA.

  View details for DOI 10.1007/s00246-016-1372-0

  View details for Web of Science ID 000377722400016

  View details for PubMedID 27064094

• Retrograde Transcatheter Device Closure of a Complex Paravalvular Leak After Bioprosthetic Pulmonary Valve Replacement in a Pediatric Patient CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Chikkabyrappa, S., Mosca, R. S., McElhinney, D. B. 2016; 87 (7): 1294-1297

  Abstract

  We report a case of retrograde transcatheter device closure of a complex paravalvular leak (PVL) after bioprosthetic pulmonary valve replacement (PVR) in a 13-year-old patient with congenital pulmonary valve stenosis. There are prior reports of pulmonary PVL closure after PVR in adults (Seery and Slack, Congenit Heart Dis 2014;9:E19-F22), but indications for and technical considerations in PVL closure after bioprosthetic PVR, particularly in children, are not well defined. © 2015 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.26316

  View details for Web of Science ID 000379985100023

  View details for PubMedID 26514545

• Postoperative Outcomes of Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals Undergoing Reconstruction of Occluded Pulmonary Artery Branches ANNALS OF THORACIC SURGERY Asija, R., Koth, A. M., Velasquez, N., Chan, F. P., Perry, S. B., Hanley, F. L., McElhinney, D. 2016; 101 (6): 2329-2334

  Abstract

  Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to understand whether patients undergoing reconstruction and incorporation of occluded pulmonary arterial branches were at risk for worse postoperative outcomes.We performed a retrospective chart review to identify patients who underwent unifocalization or unifocalization revision with incorporation of occluded pulmonary artery branches. Patients with and without occluded branches were compared, with a focus on clinical outcomes.We studied 92 patients who underwent unifocalization procedures between 2010 and 2014, 17 (18%) of whom underwent reconstruction of occluded pulmonary artery branches. Patients with occluded vessels were more likely to require staged unifocalization procedures, although more than two thirds of this cohort eventually underwent complete intracardiac repair. Durations of mechanical ventilation, intensive care, hospital stay, and the need for early reoperation were similar between the two groups.Occluded pulmonary arterial branches can be safely recruited into the pulmonary vasculature in patients with TOF/PA/MAPCAs without a significant difference in postoperative outcomes compared with patients who did not have an occluded branch. Incorporation of occluded branches may also facilitate ultimate complete intracardiac repair in this complex population of patients.

  View details for DOI 10.1016/j.athoracsur.2015.12.049

  View details for PubMedID 26947013

• Relative Risk Factors for Cardiac Erosion Following Transcatheter Closure of Atrial Septal Defects A Case-Control Study CIRCULATION McElhinney, D. B., Quartermain, M. D., Kenny, D., Alboliras, E., Amin, Z. 2016; 133 (18): 1738-?

  Abstract

  Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors.All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion.In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

  View details for DOI 10.1161/CIRCULATIONAHA.115.019987

  View details for Web of Science ID 000375604400004

  View details for PubMedID 27002094

• Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves An International, Multicenter Registry Study CIRCULATION McElhinney, D. B., Cabalka, A. K., AboulHosn, J. A., Eicken, A., Boudjemline, Y., Schubert, S., Himbert, D., Asnes, J. D., Salizzoni, S., Bocks, M. L., Cheatham, J. P., Momenah, T. S., Kim, D. W., Schranz, D., Meadows, J., Thomson, J. D., Goldstein, B. H., Crittendon, I., Fagan, T. E., Webb, J. G., Horlick, E., Delaney, J. W., Jones, T. K., Shahanavaz, S., Moretti, C., Hainstock, M. R., Kenny, D. P., Berger, F., Rihal, C. S., Dvir, D. 2016; 133 (16): 1582-?

  Abstract

  Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports.An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type.TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

  View details for DOI 10.1161/CIRCULATIONAHA.115.019353

  View details for Web of Science ID 000374553400013

  View details for PubMedID 26994123

• Intentional Fracture of Maximally Dilated Balloon-Expandable Pulmonary Artery Stents Using Ultra-High-Pressure Balloon Angioplasty: A Preliminary Analysis. Circulation. Cardiovascular interventions Morray, B. H., McElhinney, D. B., Marshall, A. C., Porras, D. 2016; 9 (4)

  Abstract

  Treatment with endovascular stents has become increasingly common for the management of vascular stenosis in congenital heart disease. The use of stents in smaller patients has been tempered by concerns about the potential for stent expansion to accommodate somatic growth. One solution to limited stent diameter is the intentional fracture of maximally dilated stents, which can be accomplished using ultra-high-pressure (UHP) balloons.This retrospective cohort study compared procedural characteristics and adverse events between a cohort of patients with branch pulmonary artery (PA) stents who underwent stent fracture using UHP balloons and control patients who underwent UHP redilation of previously placed PA stents without stent fracture between 2004 and 2014. Two control patients were selected for every case. Thirty-three PA stents were fractured in 31 patients with a median of 10 years after initial stent placement. The median balloon:waist ratio was 1.17 (1-1.71), and the median inflation pressure was 20 (8-30) atm. There were significant reductions in pressure gradient after angioplasty, with no difference in postangioplasty gradients between cases and controls. There were no major PA complications in the stent fracture group and no difference in the number of adverse events between the 2 groups.In this small series, PA stent fracture using UHP balloon angioplasty was feasible and did not result in major complications although predictors of successful fracture were not identified. Intentional fracture with UHP balloon angioplasty may be considered when treating stents that have become restrictive despite maximal dilation.

  View details for DOI 10.1161/CIRCINTERVENTIONS.115.003281

  View details for PubMedID 27059684

• Development and Validation of a Major Adverse Transplant Event (MATE) Score to Predict Graft Loss after Pediatric Heart Transplantation Almond, C. S., Rossano, J. W., Hoen, H., Castleberzy, C., Auerbach, S., Yang, L., Lal, A. K., Everitt, M. D., Pahl, E., Fenton, M., Hollander, S. A., McElhinney, D. B., Rosenthal, D. N., Daly, K. P., Desai, M. ELSEVIER SCIENCE INC. 2016: S104

  View details for DOI 10.1016/j.healun.2016.01.285

  View details for Web of Science ID 000374718100260

• Utility of the Routine Screening Echocardiogram After Endomyocardial Biopsy for Identification of Hemopericardium or Tricuspid Valve Injury Peng, D. M., Kipps, A. K., Palmon, M., Khalapyan, T. Z., Almond, C. S., Chen, S., Hollander, S. A., Tacy, T. A., Rosenthal, D. N., McElhinney, D. ELSEVIER SCIENCE INC. 2016: S403

  View details for DOI 10.1016/j.healun.2016.01.1162

  View details for Web of Science ID 000374718101476

• Relationship between Hemodynamic Variables and NYHA/Ross Functional Capacity in Children with Advanced Heart Failure Dykes, J. C., Chen, S., Lin, A., Rosenthal, D. N., Maeda, K., McElhinney, D. B., Almond, C. S. ELSEVIER SCIENCE INC. 2016: S416–S417

  View details for DOI 10.1016/j.healun.2016.01.1202

  View details for Web of Science ID 000374718101514

• Hemodynamic Findings Associated with End-Organ Dysfunction in Children with End-Stage Heart Failure Chen, S., McElhinney, D. B., Dykes, J. C., Rosenthal, D. N., Hollander, S. A., Maeda, K., Jensen, K., Yarlagadda, V. V., Ford, M. A., Price, J. F., Almond, C. S. ELSEVIER SCIENCE INC. 2016: S414

  View details for DOI 10.1016/j.healun.2016.01.1195

  View details for Web of Science ID 000374718101507

• Measuring the Effect of Dipyridamole and Clopidogrel Using Thromboelastography/Platelet Mapping (R): Is There Evidence of a Meaningful Dose-Response Relationship in Children Supported with the Berlin Heart EXCOR Ventricular Assist Device? May, L. J., Lo, C., Tesoro, T. M., Zehnder, J. L., Yang, L., Chen, S., Lee, J., McElhinney, D. B., Rosenthal, D. N., Maeda, K., Almond, C. S. ELSEVIER SCIENCE INC. 2016: S47

  View details for DOI 10.1016/j.healun.2016.01.127

  View details for Web of Science ID 000374718100104

• Aortic Root Distortion and Aortic Insufficiency During Balloon Angioplasty of the Right Ventricular Outflow Tract Prior to Transcatheter Pulmonary Valve Replacement. Journal of interventional cardiology Torres, A. J., McElhinney, D. B., Anderson, B. R., Turner, M. E., Crystal, M. A., Timchak, D. M., Vincent, J. A. 2016; 29 (2): 197-207

  Abstract

  To describe the significance of aortic root distortion (AD) and/or aortic valve insufficiency (AI) during balloon angioplasty of the right ventricular outflow tract (RVOT) performed to rule out coronary artery compression prior to transcatheter pulmonary valve (TPV) implantation.AD/AI was assessed by retrospective review of all procedural aortographies performed to evaluate coronary anatomy prior to TPV implantation. AD/AI was also reviewed in all pre-post MPV implant echocardiograms to assess for progression.From 04/2007 to 3/2015, 118 pts underwent catheterization with intent for TPV implant. Mean age and weight were 24.5 ± 12 years and 64.3 ± 20 kg, respectively. Diagnoses were: TOF (53%), D-TGA/DORV (18%), s/p Ross (15%), and Truncus (9%). Types of RV-PA connections were: conduits (96), bioprosthetic valves (14), and other (7). Successful TPV implant occurred in 91 pts (77%). RVOT balloon angioplasty was performed in 43/118 pts (36%). Aortography was performed in 18/43 pts with AD/AI noted in 6/18 (33%); 2 with D-TGA (1 s/p Lecompte, 1 s/p Rastelli), 2 with TOF, 1 Truncus and 1 s/p Ross. Procedure was aborted in the 2 who developed severe AD/AI. TPV was implanted in 3/4 patients with mild AD/AI. Review of pre-post TPV implantation echocardiograms in 83/91 pts (91%) revealed no new/worsened AI in any patient.AD/AI is relatively common on aortography during simultaneous RVOT balloon angioplasty. Lack of AI progression by echocardiography post-TPV implant suggests these may be benign findings in most cases. However, AD/AI should be carefully evaluated in certain anatomic subtypes with close RVOT/aortic alignments. (J Interven Cardiol 2016;29:197-207).

  View details for DOI 10.1111/joic.12270

  View details for PubMedID 26822282

• Exploring the Role of Polycythemia in Patients With Cyanosis After Palliative Congenital Heart Surgery. Pediatric critical care medicine Siehr, S. L., Shi, S., Hao, S., Hu, Z., Jin, B., Hanley, F., Reddy, V. M., McElhinney, D. B., Ling, X. B., Shin, A. Y. 2016; 17 (3): 216-222

  Abstract

  To understand the relationship between polycythemia and clinical outcome in patients with hypoplastic left heart syndrome following the Norwood operation.A retrospective, single-center cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Infants with hypoplastic left heart syndrome admitted to our medical center from September 2009 to December 2012 undergoing stage 1/Norwood operation.None.Baseline demographic and clinical information including first recorded postoperative hematocrit and subsequent mean, median, and nadir hematocrits during the first 72 hours postoperatively were recorded. The primary outcomes were in-hospital mortality and length of hospitalization. Thirty-two patients were included in the analysis. Patients did not differ by operative factors (cardiopulmonary bypass time and cross-clamp time) or traditional markers of severity of illness (vasoactive inotrope score, lactate, saturation, and PaO2/FIO2 ratio). Early polycythemia (hematocrit value > 49%) was associated with longer cardiovascular ICU stay (51.0 [± 38.6] vs 21.4 [± 16.2] d; p < 0.01) and total hospital length of stay (65.0 [± 46.5] vs 36.1 [± 20.0] d; p = 0.03). In a multivariable analysis, polycythemia remained independently associated with the length of hospitalization after controlling for the amount of RBC transfusion (weight, 4.36 [95% CI, 1.35-7.37]; p < 0.01). No difference in in-hospital mortality rates was detected between the two groups (17.6% vs 20%).Early polycythemia following the Norwood operation is associated with longer length of hospitalization even after controlling for blood cell transfusion practices. We hypothesize that polycythemia may be caused by hemoconcentration and used as an early marker of capillary leak syndrome.

  View details for DOI 10.1097/PCC.0000000000000654

  View details for PubMedID 26825044

• SCAI Is the voice of congenital and structural heart disease interventionalists CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Kavinsky, C. J., McElhinney, D. B., Blankenship, J. C. 2016; 87 (3): 349–50

  View details for PubMedID 26919335

• The Archaeology of Uncommon Interventions: Articulating the Rationale for Transcatheter Closure of Congenital Coronary Artery Fistulas in Asymptomatic Children CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2016; 87 (3): 419–20

  Abstract

  Transcatheter closure of coronary artery fistulas can be executed successfully in infants and children with few serious procedural complications. Indications for and long-term outcomes of closure of coronary artery fistulas remain poorly defined. Registries may offer the best opportunity for advancing our knowledge about uncommon interventions such as coil or device closure of coronary artery fistulas, but to do so, they must include sufficient data and evaluate factors potentially associated with salutary or adverse outcome.

  View details for PubMedID 26919340

• Recapitulation and Synthesis Proliferation of Meta-Analysis in Structural Interventional Cardiology CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2016; 9 (2): e003623

  View details for PubMedID 26848129

• Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions. Congenital heart disease Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-87

  Abstract

  Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.

  View details for DOI 10.1111/chd.12290

  View details for PubMedID 26219731

• Exploring Value in Congenital Heart Disease: An Evaluation of Inpatient Admissions CONGENITAL HEART DISEASE Shin, A. Y., Hu, Z., Jin, B., Lal, S., Rosenthal, D. N., Efron, B., Sharek, P. J., Sutherland, S. M., Cohen, H. J., McElhinney, D. B., Roth, S. J., Ling, X. B. 2015; 10 (6): E278-E287

  Abstract

  Understanding value provides an important context for improvement. However, most health care models fail to measure value. Our objective was to categorize inpatient encounters within an academic congenital heart program based on clinical outcome and the cost to achieve the outcome (value). We aimed to describe clinical and nonclinical features associated with value.We defined hospital encounters based on outcome per resource utilized. We performed principal component and cluster analysis to classify encounters based on mortality, length of stay, hospital cost and revenue into six classes. We used nearest shrunken centroid to identify discriminant features associated with the cluster-derived classes. These features underwent hierarchical clustering and multivariate analysis to identify features associated with each class.We analyzed all patients admitted to an academic congenital heart program between September 1, 2009, and December 31, 2012.A total of 2658 encounters occurred during the study period. Six classes were categorized by value. Low-performing value classes were associated with greater institutional reward; however, encounters with higher-performing value were associated with a loss in profitability. Encounters that included insertion of a pediatric ventricular assist device (log OR 2.5 [95% CI, 1.78 to 3.43]) and acquisition of a hospital-acquired infection (log OR 1.42 [95% CI, 0.99 to 1.87]) were risk factors for inferior health care value.Among the patients in our study, institutional reward was not associated with value. We describe a framework to target quality improvement and resource management efforts that can benefit patients, institutions, and payers alike.

  View details for DOI 10.1111/chd.12290

  View details for Web of Science ID 000367379300004

• Iatrogenic aortopulmonary communications after transcatheter interventions on the right ventricular outflow tract or pulmonary artery: Pathophysiologic, diagnostic, and management considerations CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Torres, A., Sanders, S. P., Vincent, J. A., El-Said, H. G., Leahy, R. A., Padera, R. F., McElhinney, D. B. 2015; 86 (3): 438-452

  Abstract

  To investigate the spectrum, etiology, and management of traumatic aortopulmonary (AP) communications after transcatheter interventions on the pulmonary circulation.An iatrogenic AP communication is an unusual complication after balloon pulmonary artery (PA) angioplasty or stenting, or transcatheter pulmonary valve replacement (TPVR). However, with the increasing application of transcatheter therapies for postoperative PA stenosis and right ventricular outflow tract (RVOT) dysfunction, including percutaneous pulmonary valve replacement, consideration of the etiology, diagnosis, and management of this problem is important for interventional cardiologists performing such procedures.We present three new cases, as well as gross anatomy and histopathology data, related to AP communications after PA interventions. We also review the literature relevant to this topic. Including these new cases, there have been 18 reported cases of iatrogenic AP communication after transcatheter interventions on the PAs or RVOT, primarily patients with transposition of the great arteries who underwent PA angioplasty after an arterial switch operation, or after TPVR in patients who had undergone a Ross procedure. The likely cause of such defects is PA trauma plus distortion of the neo-aortic anastomosis resulting from angioplasty or stenting of the RVOT or central PAs, with subsequent dissection through the extravascular connective tissue and into the closely adjacent vessel through the devitalized tissue at the anastomosis.Cardiologists performing PA or RVOT interventions should be aware of the possibility of a traumatic AP communication and consider this diagnosis when confronted with suggestive signs and symptoms. © 2015 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.25897

  View details for Web of Science ID 000359811600022

  View details for PubMedID 25676815

• Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation CIRCULATION-CARDIOVASCULAR INTERVENTIONS Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)

  Abstract

  Aortic wall complications can occur in unrepaired aortic coarctation (CoA) and after surgical repair or endovascular treatment. This review summarizes the available literature and current understanding of aortic wall injury (AWI) surrounding the management of CoA, focusing specifically on acute and follow-up AWI after endovascular treatment. There have been 23 reported cases of aortic rupture after endovascular treatment for CoA, including angioplasty alone, bare metal stenting, and primary covered stent therapy. Even if these published cases represent only a minority of ruptures that have actually occurred, the incidence is substantially <1%. The incidence of acute aneurysm formation was 0% to 13% after angioplasty, 0% to 5% after bare metal stent placement, and <1% after covered stent placement. The reported incidence and natural history of both acute and new AWI during follow-up after endovascular therapy for CoA varies considerably, likely secondary to ascertainment and reporting biases and inconsistent definitions. Although important AWI after endovascular treatment of CoA seems to be declining in frequency with increasing experience and improving technology, it remains one of the most important potential adverse outcomes. Long-term surveillance for new AWI and monitoring of existing AWI is mandatory, with institution of appropriate treatment when necessary. A central research focus in this population should be determination of the appropriate treatment for both native and recurrent CoA across various ages with regard to limiting recurrent CoA and preventing associated aortic wall complications, in addition to determining the appropriate treatment of various AWI. Consistent definitions and reporting are necessary to truly understand the incidence of, risk factors for, and measures protective against AWI after angioplasty or stent implantation for CoA.

  View details for DOI 10.1161/CIRCINTERVENTIONS.115.002840

  View details for Web of Science ID 000209979100006

• Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure ANNALS OF THORACIC SURGERY Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Sondergaard, L., Jones, T. K. 2015; 100 (3): 996-1003

  Abstract

  Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure.Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials.Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p < 0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n = 8) or surgical (n = 6) TPV reinterventions for obstruction with stent fracture (n = 9), endocarditis with conduit obstruction (n = 3), or reoperation (n = 2). Freedom from TPV explant was 89% ± 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up.The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

  View details for DOI 10.1016/j.athoracsur.2015.04.108

  View details for Web of Science ID 000360962600041

  View details for PubMedID 26190388

• Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction After the Ross Procedure. Annals of thoracic surgery Gillespie, M. J., McElhinney, D. B., Kreutzer, J., Hellenbrand, W. E., El-Said, H., Ewert, P., Rhodes, J. F., Søndergaard, L., Jones, T. K. 2015; 100 (3): 996-1002

  Abstract

  Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure.Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials.Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p < 0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n = 8) or surgical (n = 6) TPV reinterventions for obstruction with stent fracture (n = 9), endocarditis with conduit obstruction (n = 3), or reoperation (n = 2). Freedom from TPV explant was 89% ± 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up.The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

  View details for DOI 10.1016/j.athoracsur.2015.04.108

  View details for PubMedID 26190388

• Aortic Wall Injury Related to Endovascular Therapy for Aortic Coarctation. Circulation. Cardiovascular interventions Tretter, J. T., Jones, T. K., McElhinney, D. B. 2015; 8 (9)

  Abstract

  Aortic wall complications can occur in unrepaired aortic coarctation (CoA) and after surgical repair or endovascular treatment. This review summarizes the available literature and current understanding of aortic wall injury (AWI) surrounding the management of CoA, focusing specifically on acute and follow-up AWI after endovascular treatment. There have been 23 reported cases of aortic rupture after endovascular treatment for CoA, including angioplasty alone, bare metal stenting, and primary covered stent therapy. Even if these published cases represent only a minority of ruptures that have actually occurred, the incidence is substantially <1%. The incidence of acute aneurysm formation was 0% to 13% after angioplasty, 0% to 5% after bare metal stent placement, and <1% after covered stent placement. The reported incidence and natural history of both acute and new AWI during follow-up after endovascular therapy for CoA varies considerably, likely secondary to ascertainment and reporting biases and inconsistent definitions. Although important AWI after endovascular treatment of CoA seems to be declining in frequency with increasing experience and improving technology, it remains one of the most important potential adverse outcomes. Long-term surveillance for new AWI and monitoring of existing AWI is mandatory, with institution of appropriate treatment when necessary. A central research focus in this population should be determination of the appropriate treatment for both native and recurrent CoA across various ages with regard to limiting recurrent CoA and preventing associated aortic wall complications, in addition to determining the appropriate treatment of various AWI. Consistent definitions and reporting are necessary to truly understand the incidence of, risk factors for, and measures protective against AWI after angioplasty or stent implantation for CoA.

  View details for DOI 10.1161/CIRCINTERVENTIONS.115.002840

  View details for PubMedID 26291468

• Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era A Multicenter Study CIRCULATION Freud, L. R., Escobar-Diaz, M. C., Kalish, B. T., Komarlu, R., Puchalski, M. D., Jaeggi, E. T., Szwast, A. L., Freire, G., Levasseur, S. M., Kavanaugh-McHugh, A., Michelfelder, E. C., Moon-Grady, A. J., Donofrio, M. T., Howley, L. W., Tierney, E. S., Cuneo, B. F., Morris, S. A., Pruetz, J. D., van der Velde, M. E., Kovalchin, J. P., Ikemba, C. M., Vernon, M. M., Samai, C., Satou, G. M., Gotteiner, N. L., Phoon, C. K., Silverman, N. H., McElhinney, D. B., Tworetzky, W. 2015; 132 (6): 481-489

  Abstract

  Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era.Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001).In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

  View details for DOI 10.1161/CIRCULATIONAHA.115.015839

  View details for Web of Science ID 000359666500003

  View details for PubMedID 26059011

• Preliminary Assessment of Tricuspid Valve Annular Velocity Parameters by Cardiac Magnetic Resonance Imaging in Adults with a Volume-Overloaded Right Ventricle: Comparison of Unrepaired Atrial Septal Defect and Repaired Tetralogy of Fallot PEDIATRIC CARDIOLOGY Ito, S., McElhinney, D. B., Adams, R., Bhatla, P., Chung, S., Axel, L. 2015; 36 (6): 1294-1300

  Abstract

  The aim is to compare tricuspid valve (TV) atrioventricular junction (AVJ) annular motion parameters in unrepaired atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) by cardiac magnetic resonance (CMR) imaging. We retrospectively reviewed CMR studies performed between November 2007 and November 2013 in patients 16-45 years of age with unrepaired ASD (with or without partial anomalous pulmonary venous return) and with repaired TOF, who had previous infundibulotomy, but have not undergone pulmonary valve replacement. Longitudinal motion of lateral TV in four-chamber view cine image was tracked through the cardiac cycle with custom software. Twenty TOF patients and 12 ASD patients were included, and values were compared with 80 controls. Right ventricular end-diastolic volume index and right ventricular end-systolic volume index were similar in the ASD and TOF groups and were significantly higher in both groups than in controls. Maximum displacement of the TV in systole, velocity at half-maximal displacement during systole, and velocity at half-maximal displacement during early diastole were all significantly lower in the TOF group than the ASD group [1.39 ± 0.47 vs. 2.21 ± 0.46 (cm, p < 0.01), 5.9 ± 2.1 vs. 10.1 ± 2.3 (cm/s, p < 0.01), and 7.7 ± 2.6 vs. 10.9 ± 3.1 (cm/s, p < 0.05)]. TOF patients have diminished early diastolic TV AVJ velocity compared to patients with an unrepaired ASD, despite similar RV volumes. This observation could suggest diastolic dysfunction or cardiac mechanics unique to the postoperative, volume-overloaded right ventricle in patients with repaired TOF.

  View details for DOI 10.1007/s00246-015-1160-2

  View details for Web of Science ID 000357683800026

  View details for PubMedID 25835201

• Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation Cheatham, J. P., Hellenbrand, W. E., Zahn, E. M., Jones, T. K., Berman, D. P., Vincent, J. A., McElhinney, D. B. 2015; 131 (22): 1960-1970

  Abstract

  Studies of transcatheter pulmonary valve (TPV) replacement with the Melody valve have demonstrated good short-term outcomes, but there are no published long-term follow-up data.The US Investigational Device Exemption trial prospectively enrolled 171 pediatric and adult patients (median age, 19 years) with right ventricular outflow tract conduit obstruction or regurgitation. The 148 patients who received and were discharged with a TPV were followed up annually according to a standardized protocol. During a median follow-up of 4.5 years (range, 0.4-7 years), 32 patients underwent right ventricular outflow tract reintervention for obstruction (n=27, with stent fracture in 22), endocarditis (n=3, 2 with stenosis and 1 with pulmonary regurgitation), or right ventricular dysfunction (n=2). Eleven patients had the TPV explanted as an initial or second reintervention. Five-year freedom from reintervention and explantation was 76±4% and 92±3%, respectively. A conduit prestent and lower discharge right ventricular outflow tract gradient were associated with longer freedom from reintervention. In the 113 patients who were alive and reintervention free, the follow-up gradient (median, 4.5 years after implantation) was unchanged from early post-TPV replacement, and all but 1 patient had mild or less pulmonary regurgitation. Almost all patients were in New York Heart Association class I or II. More severely impaired baseline spirometry was associated with a lower likelihood of improvement in exercise function after TPV replacement.TPV replacement with the Melody valve provided good hemodynamic and clinical outcomes up to 7 years after implantation. Primary valve failure was rare. The main cause of TPV dysfunction was stenosis related to stent fracture, which was uncommon once prestenting became more widely adopted.URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

  View details for DOI 10.1161/CIRCULATIONAHA.114.013588

  View details for PubMedID 25944758

• Intermediate Outcomes in the Prospective, Multicenter Coarctation of the Aorta Stent Trial (COAST) CIRCULATION Meadows, J., Minahan, M., McElhinney, D. B., McEnaney, K., Ringel, R. 2015; 131 (19): 1656-1664

  Abstract

  The Coarctation of the Aorta Stent Trial (COAST) was designed to assess the safety and efficacy of the Cheatham Platinum stent when used in children and adults with native or recurrent coarctation. Acute outcomes have been reported. We report here follow-up to 2 years.A total of 105 patients underwent attempted implantation, with 104 successes. There were no procedural deaths, serious adverse events, or surgical intervention. All patients experienced immediate reduction in upper- to lower-extremity blood pressure difference with sustained improvement to 2 years. Rates of hypertension and medication use decreased from baseline to 12 months and remained largely unchanged at 2 years. Six aortic aneurysms have been identified: 5 were successfully treated with covered stent placement, and 1 resolved without intervention. Stent fractures were noted in 2 patients at 1 year and 11 patients at 2 years, with evidence of fracture progression. To date, only larger stent diameter was associated with stent fracture. Twelve additional fractures have occurred after 2 years. No fracture has resulted in loss of stent integrity, stent embolization, aortic wall injury, or reobstruction. Nine reinterventions occurred in the first 2 years for stent redilation and address of aneurysms, and 10 additional reinterventions occurred after 2 years.The Cheatham Platinum stent is safe and associated with persistent relief of aortic obstruction. Stent fracture and progression of fracture occur but have not resulted in clinically important sequelae. Reintervention is common and related to early and late aortic wall injury and need for re-expansion of small-diameter stents.URL: http://www.clinicaltrials.gov. Unique identifier: NCT00552812.

  View details for DOI 10.1161/CIRCULATIONAHA.114.013937

  View details for Web of Science ID 000354213000011

  View details for PubMedID 25869198

• Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hörer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)

  View details for DOI 10.1161/CIRCINTERVENTIONS.114.002155

  View details for PubMedID 25873731

• Hemodynamic Profiles in Children With End-Stage Heart Failure: Analysis of Data From the Pediatric Heart Transplant Study Group Chen, S., Gajarski, R. J., Lin, A., May, L. J., Rosenthal, D. N., Everitt, M. D., McElhinney, D. B., Shin, A. Y., Hollander, S. A., Pruitt, E., Almond, C. S. ELSEVIER SCIENCE INC. 2015: S35

  View details for DOI 10.1016/j.healun.2015.01.084

  View details for Web of Science ID 000353251500073

• The Current Role of Temporary Circulatory Assist Devices in US Children Awaiting Heart Transplant Yarlagadda, V. V., Shin, A. Y., Reinhartz, O., Palmon, M., Shuttleworth, P., Maeda, K., Yeh, J., Murray, J., Rosenthal, D. N., McElhinney, D. B., Almond, C. S. ELSEVIER SCIENCE INC. 2015: S100

  View details for DOI 10.1016/j.healun.2015.01.267

  View details for Web of Science ID 000353251500249

• Thromboelastography/Platelet Mapping (R) and Aspirin: Is There Evidence of a Meaningful Dose-Response Relationship in Children Supported With the Berlin Heart EXCOR Ventricular Assist Device? May, L. J., Lo, C., Tesoro, T. M., Zehnder, J. L., Chen, S., Lee, J., Desai, M., McElhinney, D. B., Rosenthal, D. N., Maeda, K., Almond, C. S. ELSEVIER SCIENCE INC. 2015: S77

  View details for DOI 10.1016/j.healun.2015.01.202

  View details for Web of Science ID 000353251500186

• Percutaneous tricuspid valve implantation: two-center experience with midterm results. Circulation. Cardiovascular interventions Eicken, A., Schubert, S., Hager, A., Hörer, J., McElhinney, D. B., Hess, J., Ewert, P., Berger, F. 2015; 8 (4)

  Abstract

  Severe tricuspid valve (TV) dysfunction may lead to surgical TV replacement with a biological valve prosthesis in patients with congenital heart disease. To expand the lifetime of this valve and reduce the number of surgeries, percutaneous TV implantation (PTVI) may be an effective alternative to repeated surgery. We report on our 2-center experience with PTVI.Between 2008 and 2014, 17 percutaneous valves were implanted in 16 patients with TV bioprosthesis dysfunction (9 females) from 2 centers. Median age and weight were 31.3 years (5-77.2) and 65.2 kg (17.7-107); 14 patients had congenital heart disease (univentricular heart with a right atrial to right ventricle bioprosthesis in 3, Ebstein's anomaly of the TV in 5, and other in 6), and 2 had acquired TV dysfunction. All procedures were successful (Melody n=7, Sapien 26 mm valve n=4, Sapien XT 29 mm valve n=6). One valve showed early dysfunction. It was replaced surgically and shortly after that a repeated PTVI was performed. The median duration of follow-up was 2.1 years (3 days to 6.3 years). The percutaneous valve was performing well in 15 of 16 patients.PTVI was safe and effectively improved TV function in all but 1 patient at midterm follow-up. We think that PTVI is a good alternative to repeated surgical TV replacements and that it may reduce the total number of open heart surgeries in these patients.

  View details for DOI 10.1161/CIRCINTERVENTIONS.114.002155

  View details for PubMedID 25873731

• Tricuspid Valve Regurgitation in Congenitally Corrected Transposition of the Great Arteries and a Left Ventricle to Pulmonary Artery Conduit ANNALS OF THORACIC SURGERY Buber, J., McElhinney, D. B., Valente, A. M., Marshall, A. C., Landzberg, M. J. 2015; 99 (4): 1348-1356

  Abstract

  The configuration of the interventricular septum can affect the function of the tricuspid valve in patients with congenitally corrected transposition of the great arteries who have a systemically functioning right ventricle. Altering septal configuration by addressing a dysfunctional conduit placed between the left ventricle (LV) and the pulmonary artery (PA) in these patients can impact septal configuration and competency of the tricuspid valve.In 38 patients with an LV to PA conduit, we evaluated relationships between conduit function, RV geometry, and tricuspid valve function, and compared these variables before and after conduit intervention.Median age at conduit implant was 4.5 years (0.5 to 36) and median total follow-up was 12 years (2 to 22). Of the 38 patients, 23 (60%) underwent conduit intervention, a median of 7.5 years after implant. In 15 of these patients (65%) the degree of tricuspid regurgitation (TR) worsened, compared with only 2 patients (15%) in the non-intervention group (p < 0.001). Worsening TR was associated with the degree of change in RV and LV ventricular diameters, change in tricuspid annulus size and tethering distance, and the degree of septal shift, as reflected by the right ventricular sphericity index (all p ≤ 0.04). In 8 of 15 patients with more severe TR at follow-up, there was also progressive RV dysfunction.Intervention for LV to PA conduit dysfunction may result in worsening TR and right ventricular function, likely due in part to altered septal shift due to changes in the interventricular pressure ratio. Management of LV to PA conduit dysfunction should take these findings into account.

  View details for DOI 10.1016/j.athoracsur.2014.11.008

  View details for Web of Science ID 000352162100045

  View details for PubMedID 25661908

• Low rate of prenatal diagnosis among neonates with critical aortic stenosis: insight into the natural history in utero ULTRASOUND IN OBSTETRICS & GYNECOLOGY Freud, L. R., Moon-Grady, A., Escobar-Diaz, M. C., Gotteiner, N. L., Young, L. T., McElhinney, D. B., Tworetzky, W. 2015; 45 (3): 326-332

  Abstract

  To better understand the natural history and spectrum of fetal aortic stenosis (AS), we aimed to (1) determine the prenatal diagnosis rate of neonates with critical AS and a biventricular (BV) outcome, and (2) describe the findings at fetal echocardiography in patients diagnosed prenatally.A multicenter, retrospective study was performed on neonates who presented with critical AS and who were discharged with a BV outcome from 2000 to 2013. The prenatal diagnosis rate was compared with that reported for hypoplastic left heart syndrome (HLHS). We reviewed fetal echocardiographic findings in patients who were diagnosed prenatally.In only 10 (8.5%) of 117 neonates with critical AS and a BV outcome was the diagnosis made prenatally, a rate significantly lower than that for HLHS in the contemporary era (82%; P < 0.0001). Of the 10 patients diagnosed prenatally, all had developed left ventricular dysfunction by a median gestational age of 33 (range, 28-35) weeks. When present, Doppler abnormalities such as retrograde flow in the aortic arch (n = 2), monophasic mitral inflow (n = 3) and left-to-right flow across the foramen ovale (n = 8) developed late in gestation (median 33 weeks).The prenatal diagnosis rate of critical AS and a BV outcome among neonates is very low, probably owing to a relatively normal four-chamber view in mid-gestation with development of significant obstruction in the third trimester. The natural history contrasts with that of severe mid-gestation AS with evolving HLHS and suggests that the gestational timing of development of significant AS has an important impact on subsequent left-heart growth in utero.

  View details for DOI 10.1002/uog.14667

  View details for Web of Science ID 000350641200014

  View details for PubMedID 25251721

• Comparison of Reported Outcomes With Percutaneous Versus Surgical Closure of Ruptured Sinus of Valsalva Aneurysm AMERICAN JOURNAL OF CARDIOLOGY Kuriakose, E. M., Bhatla, P., McElhinney, D. B. 2015; 115 (3): 392-398

  Abstract

  Sinus of Valsalva aneurysm is a rare cardiac malformation that stems from incomplete fusion of the aortic media and the aortic valve annulus, a weakness that may result in rupture of the sinus, large left-to-right shunt, and severe congestive heart failure. Historically, this lesion has been repaired surgically, but percutaneous closure (PC) has emerged as a therapeutic intervention over the last 20 years. We review and contrast 34 studies detailing the PC approach with 16 studies on surgical closure (SC), together comprising a total of 877 patients who were treated for ruptured sinus of Valsalva aneurysm from 1956 to 2014. Both groups had similar sites of rupture, age distribution, and clinical symptoms at presentation. Selection bias ultimately prohibits a direct comparison between the 2 groups as patients who underwent SC often had worse aortic regurgitation and more complex associated lesions, including endocarditis, bicuspid aortic valve, tunnel-type fistulous connections, larger defect size, and multiple site of rupture. In conclusion, although SC is indicated and reserved for these more complicated patients, our review of previously published reports reveals that PC in patients who are too ill to undergo bypass, with mild or no aortic regurgitation and simple associated defects (muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus), can be safe, effective, and practical.

  View details for DOI 10.1016/j.amjcard.2014.11.013

  View details for Web of Science ID 000348629400019

  View details for PubMedID 25488356

• First-in-Man Primacy and the Nexus of Innovation in Interventional Cardiology CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B. 2015; 8 (2): e002406

  View details for DOI 10.1161/CIRCINTERVENTIONS.115.002406

  View details for Web of Science ID 000349804000001

  View details for PubMedID 25657318

• Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)

  View details for DOI 10.1161/JAHA.114.001076

  View details for Web of Science ID 000345067600011

  View details for PubMedCentralID PMC4338694

• Management of cardiovascular risk factors in adults with congenital heart disease. Journal of the American Heart Association Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)

  View details for DOI 10.1161/JAHA.114.001076

  View details for PubMedID 25359401

• Pulmonary Deadspace and Postoperative Outcomes in Neonates Undergoing Stage 1 Palliation Operation for Single Ventricle Heart Disease PEDIATRIC CRITICAL CARE MEDICINE Shakti, D., McElhinney, D. B., Gauvreau, K., Yarlagadda, V. V., Laussen, P. C., Betit, P., Myrer, M. L., Thiagarajan, R. R. 2014; 15 (8): 728-734

  Abstract

  Increased pulmonary dead space fraction (VD/VT) has been associated with prolonged mechanical ventilation after surgery for congenital heart disease. The association of VD/VT with clinical outcomes in neonates undergoing stage 1 palliation for single ventricle congenital heart disease has not been reported. We describe changes in VD/VT, differences in VD/VT based on shunt type (right ventricle to pulmonary artery conduit vs modified Blalock-Taussing shunt) and association of VD/VT with postoperative outcomes in patients undergoing stage 1 palliation.Retrospective chart review for demographic, hemodynamics, outcome information, and VD/VT values were collected at 6-hour intervals during the first 48 postoperative hours in neonates undergoing stage 1 palliation. VD/VT was calculated using mixed expired CO2 (PeCO2) obtained from capnography and paired arterial blood gas CO2 values.Cardiac ICU in a tertiary care pediatric hospital.Newborns with single ventricle congenital heart disease undergoing stage 1 palliation during 2003-2004.Of the 51 patients, 31 had right ventricle to pulmonary artery and 20 had Blalock-Taussing shunt. Although VD/VT was lower in the Blalock-Taussing shunt group over all time points (p = 0.02), maximal VD/VT on day 1 (0.49 ± 0.07) and on day 2 (0.46 ± 0.08) were not different between the shunt groups. VD/VT decreased significantly over time in both shunt groups (p = 0.001 for right ventricle to pulmonary artery; p < 0.001 for Blalock-Taussing shunt). Higher maximal VD/VT during first 48 postoperative hours was independently associated with fewer ventilator (β = -26.6; p = 0.035) and hospital-free days in the first month after stage 1 palliation (β = -40.4; p = 0.002) after adjusting for potential confounders in a multivariable linear regression model.Increased pulmonary dead space exists early after stage 1 palliation operation for single ventricle congenital heart disease. Higher VD/VT during the first 48 postoperative hours was associated with longer duration of ventilation and hospital LOS and may be a useful marker of postoperative outcomes in this population.

  View details for DOI 10.1097/PCC.0000000000000226

  View details for Web of Science ID 000343049200010

  View details for PubMedID 25072474

• Durability of large diameter right ventricular outflow tract conduits in adults with congenital heart disease INTERNATIONAL JOURNAL OF CARDIOLOGY Buber, J., Assenza, G. E., Huang, A., Valente, A. M., Emani, S. M., Gauvreau, K., Marshal, A. C., McElhinney, D. B., Landzberg, M. J. 2014; 175 (3): 455-463

  Abstract

  Subpulmonary ventricular outflow conduits are utilized routinely to repair complex congenital cardiac abnormalities, but are limited by the inevitable degeneration and need for reintervention. Data on conduit durability and propensity to dysfunction in the adult population are limited.The study included 288 consecutive patients ≥18 years of age who were evaluated between 1991 and 2010 after placement of a ≥18 mm conduit. Freedom from hemodynamic conduit dysfunction served as our primary outcome. Freedom from reintervention, overall mortality and heart transplantation were also evaluated.Median age at conduit implant was 19 years and median follow-up duration was 13 years. Probabilities of survival without conduit dysfunction and reintervention at 5, 10 and 15 years were 87%, 63%, and 49%, and 95%, 81%, and 56%, respectively. Smaller conduit diameter (18-20mm) was associated with lower probability of survival without dysfunction in the entire study cohort, with prominent effects in patients in both the lowest and the highest age quartiles. Other parameters with similar associations were higher BMI, native anatomy of tetralogy of Fallot or truncus arteriosus, and active smoking.Adult congenital heart disease patients with conduit diameter ≥18 mm had an approximately 50% chance of developing hemodynamic conduit dysfunction and undergoing conduit reintervention by 15 years of post-implant, and a 30% likelihood of undergoing conduit reoperation in the same time frame. The importance of these data is underscored by the increasing number of adults with congenital heart diseases seeking care and the recent advances in transcatheter valve replacement for dysfunctional conduits.

  View details for DOI 10.1016/j.ijcard.2014.06.023

  View details for Web of Science ID 000340251600015

  View details for PubMedID 25002319

• Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome Postnatal Outcomes of the First 100 Patients CIRCULATION Freud, L. R., McElhinney, D. B., Marshall, A. C., Marx, G. R., Friedman, K. G., del Nido, P. J., Emani, S. M., Lafranchi, T., Silva, V., Wilkins-Haug, L. E., Benson, C. B., Lock, J. E., Tworetzky, W. 2014; 130 (8): 638-?

  Abstract

  Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0.01). After a median follow-up of 5.4 years, freedom from cardiac death among all BV patients was 96±4% at 5 years and 84±12% at 10 years, which was better than HLHS patients (log-rank P=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, -1.3 to +8.2), and 80% had normal ejection fraction.Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted.

  View details for DOI 10.1161/CIRCULATIONAHA.114.009032

  View details for Web of Science ID 000341300100011

  View details for PubMedID 25052401

• Technical Challenges of Atrial Septal Stent Placement in Fetuses with Hypoplastic Left Heart Syndrome and Intact Atrial Septum CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Kalish, B. T., Tworetzky, W., Benson, C. B., Wilkins-Haug, L., Mizrahi-Arnaud, A., McElhinney, D. B., Lock, J. E., Marshall, A. C. 2014; 84 (1): 77-85

  Abstract

  The objective of this study was to describe our single-institution experience with prenatal atrial septal stent placement for fetuses with hypoplastic left heart syndrome and an intact atrial septum (HLHS/IAS).Infants born with HLHS/IAS are at high risk for neonatal death, despite maximal postnatal therapy. Prenatal atrial septoplasty by static balloon dilation has been effective in decompressing the left atrium (LA) in utero, but several factors have limited the size of septal defects. We attempted to overcome the limitations of balloon septoplasty using transcatheter atrial septal stents.All records from our institution of fetuses with HLHS/IAS that underwent prenatal atrial septal stent placement were reviewed, including operative notes and echocardiograms.Nine fetuses between 24 and 31 weeks gestation with HLHS/IAS underwent attempted fetal atrial septal stent placement. A stent was deployed across the atrial septum in five fetuses, with four fetuses demonstrating flow across the stent at the time of intervention. In four cases, stent placement failed due to malposition or embolization, but in three of the four cases, atrial balloon septoplasty at the same in-utero procedure successfully and acutely decompressed the LA. There were no maternal complications. There was one fetal demise. The remaining eight fetuses survived to delivery, but four died in the neonatal period (two of which had been stented).Ultrasound-guided atrial septal stent placement is feasible in some fetuses with HLHS/IAS. Visualization of the septum and catheter tip is critical to technical success. Additional experience is necessary to determine the clinical impact of this intervention. © 2013 Wiley Periodicals, Inc.

  View details for DOI 10.1002/ccd.25098

  View details for Web of Science ID 000337970100018

  View details for PubMedID 23804575

• Use and Performance of the Melody Transcatheter Pulmonary Valve in Native and Postsurgical, Nonconduit Right Ventricular Outflow Tracts CIRCULATION-CARDIOVASCULAR INTERVENTIONS Meadows, J. J., Moore, P. M., Berman, D. P., Cheatham, J. P., Cheatham, S. L., Porras, D., Gillespie, M. J., Rome, J. J., Zahn, E. M., McElhinney, D. B. 2014; 7 (3): 374-380

  Abstract

  Melody Transcatheter Pulmonary Valve (TPV) replacement therapy represents an important advance in congenital cardiovascular interventions. The off-label extension of the Melody TPV to patients with nonconduit outflow tracts (right ventricular outflow tract [RVOT]) has the potential to vastly expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. However, knowledge on the performance of the Melody TPV in this setting is limited.This is a multicenter, retrospective review of the Melody TPV when placed in nonconduit RVOTs, in which at least a portion of the circumference was composed of native tissue. Five centers contributed data on 31 patients. The median age at implantation was 24 years (range, 7-66). At a median follow-up of 15 months, all patients were alive. No patient had greater than mild TPV insufficiency, and the median maximum instantaneous gradients across the RVOT was 23 mm Hg. Stent fracture occurred in 32%. Eight patients developed more than mild TPV obstruction, of whom 6 were associated with identified stent fracture. Three patients developed blood stream infections. There were 5 reinterventions in 3 patients, including 3 repeat TPV implantations and 2 TPV explantations.Melody TPV implantation is feasible in selected patients with RVOTs comprised solely or predominantly native tissue and has the potential to expand the population of patients eligible to benefit from nonsurgical restoration of RVOT function. In early follow-up, valve competency seems preserved. The dominant mechanism of valve dysfunction seems to be related to stent fracture with recurrent obstruction. Additional data are necessary to better understand how to safely expand TPV therapy to this population.

  View details for DOI 10.1161/CIRCINTERVENTIONS.114.001225

  View details for Web of Science ID 000337746700013

  View details for PubMedID 24867892

• Feasibility and Short-Term Outcomes of Percutaneous Transcatheter Pulmonary Valve Replacement in Small (< 30 kg) Children With Dysfunctional Right Ventricular Outflow Tract Conduits CIRCULATION-CARDIOVASCULAR INTERVENTIONS Berman, D. P., McElhinney, D. B., Vincent, J. A., Hellenbrand, W. E., Zahn, E. M. 2014; 7 (2): 142-148

  Abstract

  In 2010, the Melody transcatheter pulmonary valve (TPV) received Food and Drug Administration approval for treatment of dysfunctional right ventricular outflow tract conduits in patients ≥30 kg. Limited data are available regarding use of this device in smaller patients.We evaluated technical and short-term clinical outcomes of 25 patients <30 kg (10 patients <20 kg) who underwent TPV replacement for treatment of conduit dysfunction at 3 centers. Median age and weight were 8.0 years (3.4-14.4) and 21.4 kg (13.8-29.0). The median conduit diameter at the time of surgical implant was 17 mm (12-23). Two patients did not undergo TPV implant (risk of coronary compression in 1; inability to advance the delivery sheath beyond the common femoral vein in 1). After successful TPV implant, the peak conduit gradient fell from 29±16 to 9±6 mm Hg (P<0.001), and all but 2 patients had no/trivial regurgitation (down from moderate or severe preimplant in 20). TPV implant was via the femoral vein in 17 patients, the right internal jugular vein in 4, and the left subclavian vein in 2 patients. At a median follow-up of 16 months, 1 patient underwent conduit replacement for recurrent conduit stenosis, 2 developed stent fracture requiring a second TPV, and 2 developed bacterial endocarditis treated with antibiotics, 1 of whom then underwent conduit replacement. The average Melody valve mean Doppler gradient and conduit regurgitation were unchanged from early postimplant.Percutaneous TPV replacement can be performed in small children with good procedural and early hemodynamic results in the majority of patients.

  View details for DOI 10.1161/CIRCINTERVENTIONS.113.000881

  View details for Web of Science ID 000337746200004

  View details for PubMedID 24569596

• Angioplasty of Obstructed Homograft Conduits in the Right Ventricular Outflow Tract With Ultra-Noncompliant Balloons Assessment of Therapeutic Efficacy and Conduit Tears CELL BIOLOGY INTERNATIONAL Hainstock, M. R., Marshall, A. C., Lock, J. E., McElhinney, D. B. 2014; 38 (1): 671-679

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.000073

  View details for Web of Science ID 000328511600014

• Angioplasty of Obstructed Homograft Conduits in the Right Ventricular Outflow Tract With Ultra-Noncompliant Balloons Assessment of Therapeutic Efficacy and Conduit Tears CIRCULATION-CARDIOVASCULAR INTERVENTIONS Hainstock, M. R., Marshall, A. C., Lock, J. E., McElhinney, D. B. 2013; 6 (6): 671-679

  Abstract

  Angioplasty and stent placement in right ventricle-to-pulmonary artery (RV-PA) conduits have been shown to prolong the functional lifespan of a conduit. Safety and efficacy of angioplasty of obstructed RV-PA homografts using ultra-noncompliant (UNC) or ultrahigh-pressure balloons are unknown.From 2004 to 2012, 70 patients underwent 76 procedures for angioplasty of RV-PA homografts with UNC Atlas balloons. The UNC group was compared with a partially contemporaneous control cohort of 81 patients who underwent 84 angioplasty procedures with conventional balloons. Acute hemodynamic changes after angioplasty of homografts with UNC balloons included significantly reduced RV:Ao pressure ratio (P=0.02) and right ventricular outflow tract gradients (P≤0.001). Balloon waist resolution was more frequently achieved with UNC balloons (P=0.04), and balloon rupture occurred less often (P<0.001). Conduit tears of any severity occurred in 22% of patients overall and were more common in the UNC group (P=0.001). Patients with any conduit tear had significantly greater reduction in their RV:Ao pressure ratio (P<0.001) and right ventricular outflow tract gradient (P=0.004) than those with no tear. There were 4 unconfined tears, all in the UNC group, with no acute decompensations or deaths and only 1 patient who required surgical management.RV-PA conduit tears are common in patients undergoing angioplasty, but clinically important tears, which only occurred during UNC angioplasty in this series, were uncommon. UNC balloons can be used to good effect with significant reduction in right ventricular outflow tract gradient and the RV:Ao ratio when compared with conventional balloons.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.000073

  View details for Web of Science ID 000328511300014

  View details for PubMedID 24254707

• Heterogeneity of Regional Function and Relation to Ventricular Morphology in Patients With Fontan Circulation AMERICAN JOURNAL OF CARDIOLOGY Lunze, F. I., Lunze, K., McElhinney, D. B., Colan, S. D., Gauvreau, K., Lange, P. E., Schmitt, B., Berger, F. 2013; 112 (8): 1207-1213

  Abstract

  The relation between underlying ventricular morphology and regional function in patients with Fontan circulation remains unclear. The aim of this study was to compare regional function and its heterogeneity in patients with tricuspid atresia (TA), biventricular apex-forming morphology (BiV), and controls. Nineteen patients (median age 12 years) with Fontan circulation who presented consecutively were prospectively enrolled and compared with age- and heart rate-matched controls. Most patients were in New York Heart Association class I (63%). Longitudinal systolic strain (S), systolic strain rate (SRsys), and early diastolic strain rate (SRdia) peaks were obtained from 6 ventricular segments, and a coefficient of variation by segment was calculated as a measure of regional heterogeneity. Systolic S, SRsys and SRdia peaks were decreased at the right and left lateral walls in both patient groups compared with controls (p ≤0.001 for all). Patients with TA had higher systolic S and SRsys in the middle of the right lateral wall than those with BiV morphology (p = 0.009 and p = 0.001, respectively). The mean coefficients of variation assessed by S and SRsys were similar in controls and patients with TA but lower in those with BiV than in controls and patients with TA (p <0.001 and p = 0.01, respectively). The mean coefficient of variation assessed by SRdia was greater only in patients with BiV than in controls (p = 0.001). In conclusion, patients with Fontan circulation have more heterogeneous systolic and early diastolic regional function than healthy control subjects, and patients with TA have better systolic regional function in the middle of the right lateral wall and less systolic heterogeneity than patients with BiV morphology.

  View details for DOI 10.1016/j.amjcard.2013.06.016

  View details for Web of Science ID 000325833700024

  View details for PubMedID 23927787

• Fracture of Cardiovascular Stents in Patients With Congenital Heart Disease Theoretical and Empirical Considerations CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Marshall, A. C., Schievano, S. 2013; 6 (5): 575-585

  View details for DOI 10.1161/CIRCINTERVENTIONS.113.000148

  View details for Web of Science ID 000329923100016

  View details for PubMedID 24129934

• Risk of Coronary Artery Compression Among Patients Referred for Transcatheter Pulmonary Valve Implantation A Multicenter Experience CIRCULATION-CARDIOVASCULAR INTERVENTIONS Morray, B. H., McElhinney, D. B., Cheatham, J. P., Zahn, E. M., Berman, D. P., Sullivan, P. M., Lock, J. E., Jones, T. K. 2013; 6 (5): 535-542

  Abstract

  The Melody transcatheter pulmonary valve (TPV) was approved for implantation in obstructed right ventricular outflow tract conduits in 2010 after a multicenter trial demonstrating improvements in conduit obstruction, regurgitation, and right ventricular pressure. A recognized risk and contraindication to TPV implantation is the demonstration of coronary artery (CA) compression during balloon angioplasty or stent placement in the overlying conduit. This study is the first to characterize the risk of CA compression in this population.From 2007 to 2012, 404 patients underwent 407 catheterizations for potential TPV implantation (median age, 18 years) at 4 centers. Three hundred forty-three patients (85%) underwent valve implantation. Twenty-one patients (5%) had evidence of CA compression with simultaneous right ventricular outflow tract angioplasty and CA angiography. Sixty-eight patients (17%) had abnormal CA anatomy. Fifteen of 21 (71%) patients with CA compression had abnormal CA anatomy. Eight patients with tetralogy of Fallot and 7 patients with transposition of the great arteries demonstrated compression. Of the 34 patients with tetralogy of Fallot and abnormal CA, 7 (21%) demonstrated CA compression.CA compression following TPV implantation can be catastrophic. CA compression was observed in 5% of patients during test balloon angioplasty. No patients in this study developed clinically apparent CA compression after TPV implantation. CA compression was significantly associated with the presence of abnormal CA anatomy, especially in patients with tetralogy of Fallot or transposition of the great arteries. Preimplantation coronary angiography with simultaneous test angioplasty is an important step to evaluate for the presence of CA compression during TPV implantation.

  View details for DOI 10.1161/CIRCINTERVENTIONS.113.000202

  View details for Web of Science ID 000329923100011

  View details for PubMedID 24065444

• Relationship between procedural adverse events associated with cardiac catheterization for congenital heart disease and operator factors: Results of a multi-institutional registry (C3PO) CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Holzer, R. J., Gauvreau, K., Kreutzer, J., Moore, J. W., McElhinney, D. B., Bergersen, L. 2013; 82 (3): 463-473

  Abstract

  Data examining the effect of operator years in practice and volume on adverse events (AE) after cardiac catheterization in patients with congenital heart disease is limited.Data were prospectively collected using a multi-center registry (C3PO). 10,885 catheterizations performed between 02/07 and 06/10 at eight institutions were included. AE rates were risk-adjusted for hemodynamic vulnerability, procedure type risk group, and age and compared between operators with different years in practice (YIP) and volume. AE occurred in 13% of procedures. Operators with less than five YIP had higher adjusted odds of any AE (OR 1.42, 95% CI 1.14-1.77) or a high severity AE (OR 1.35, 95% CI 1.04-1.75), when compared with operators with 5 to less than 25 YIP (5 < 25), while operators with ≥25 YIP had higher odds of a high severity (but not any) AE (OR 1.39, 95% CI 1.08-1.80). Operators with <5 YIP had a higher percentage of preventable AE (out of all AE, 16% vs. 8%, P < 0.001) as well as higher odds of vascular or cardiac trauma (OR 1.81, 95% CI 1.11-2.97), or technical AE (OR 1.98, 95% CI 1.31-2.99) when compared with operators with 5 < 25 YIP. There was no consistent relationship between operator volume, and incidence of AE.Operators with less than 5 years in practice have higher risk-adjusted AE rates. While an important consideration in guiding and mentoring operators with fewer years in practice, it is important to emphasize that reporting adverse events does not take into account procedural efficacy.

  View details for DOI 10.1002/ccd.24866

  View details for Web of Science ID 000323454100047

  View details for PubMedID 23413194

• Outcomes After Primary Transcatheter Therapy in Infants and Young Children With Severe Bilateral Peripheral Pulmonary Artery Stenosis CIRCULATION-CARDIOVASCULAR INTERVENTIONS Cunningham, J. W., McElhinney, D. B., Gauvreau, K., Bergersen, L., Lacro, R. V., Marshall, A. C., Smoot, L., Lock, J. E. 2013; 6 (4): 460-467

  Abstract

  Angioplasty and stent implantation have become accepted therapies for isolated peripheral pulmonary stenosis, and have been shown to increase vessel diameter and reduce right ventricular (RV) pressure acutely in patients with pulmonary artery (PA) stenosis. The purpose of this study was to assess long-term outcomes after primary transcatheter therapy for peripheral pulmonary stenosis.We studied 69 patients who underwent primary transcatheter intervention for severe isolated peripheral pulmonary stenosis at ≤ 5 years of age. Genetic/syndromic diagnoses included Williams syndrome (n=23), non-Williams familial arteriopathy (n=12), and Alagille syndrome (n=3). At the initial PA intervention, median RV:aortic pressure ratio decreased from 1.00 to 0.88 (median decrease, 0.18; P<0.001). Patients with a higher preintervention RV:aortic pressure ratio had a greater reduction (P<0.001). During follow-up (median, 8.5 years), 10 patients died, 5 from complications of PA catheterization (all before 1998). Thirteen patients underwent surgical PA intervention, most within 1 year and along with repair of supravalvar aortic stenosis. Freedom from any PA reintervention was 38 ± 6% at 1 year and 22 ± 6% at 5 years. The median RV:aortic pressure ratio decreased from 1.0 at baseline to 0.53 at the most recent catheterization (P<0.001), and 82% of patients with available clinical follow-up were asymptomatic.Transcatheter therapy for infants with severe peripheral pulmonary stenosis has become safer, regardless of genetic condition. Coupled with reintervention and surgical relief in selected cases, RV:aortic pressure ratios decrease substantially and most patients are asymptomatic at late follow-up.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.000061

  View details for Web of Science ID 000323323300021

  View details for PubMedID 23941859

• Echocardiographic predictors of left ventricular dysfunction after aortic valve surgery in children with chronic aortic regurgitation. Congenital heart disease Selamet Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315

  Abstract

  OBJECTIVE: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation. METHODS: Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2. RESULTS: Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve. CONCLUSION: Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

  View details for DOI 10.1111/chd.12009

  View details for PubMedID 23075071

• Wire-related Pulmonary Artery Injury during Pediatric and Adult Congenital Interventional Cardiac Catheterization CONGENITAL HEART DISEASE Esch, J. J., Bergersen, L., McElhinney, D. B., Porras, D., Lock, J. E., Marshall, A. C. 2013; 8 (4): 296-301

  Abstract

  Guidewires used in pediatric catheterization are typically floppy, soft, or J-tipped, and are generally assumed to be atraumatic. A recent sentinel case suggested that such wires may cause clinically significant pulmonary artery (PA) injury. We sought to determine the incidence of wire-related PA injury as a cause of "idiopathic" airway bleeding (endobronchial blood) during interventional cardiac catheterization in patients with congenital heart disease.The Children's Hospital Boston database of cardiac catheterizations was reviewed for adverse events (AEs) indicating possible PA injury occurring between September 2006 and August 2011. Procedure notes were reviewed, and when the clinical scenario was suggestive of wire injury or was not clear, relevant angiograms were reviewed.One thousand forty-seven cases involving PA dilation were performed in the period of interest. Five cases of probable wire injury were identified, suggesting an incidence of approximately 0.5 per 100 cases. Of these five cases, trauma was judged due to floppy-tipped wires in two, soft-tipped wires in two, and a J-tipped wire in one. In three cases, the distal wire was looped such that the leading segment was stiffer than the wire tip. Clinical manifestations of wire injury comprised contrast within the airway, vessel aneurysm/tear, obstructive intimal flap, blood from the endotracheal tube, hemothorax, and wedge defect on lung scan. These injuries were relatively benign and did not result in instability or prolonged bleeding.Wire injury to the PAs is relatively uncommon, although possible with even floppy-tipped wires. The mechanism and implications of such injuries are markedly different than balloon-mediated vascular tears.

  View details for DOI 10.1111/chd.12015

  View details for Web of Science ID 000326501000012

  View details for PubMedID 23075214

• Echocardiographic Predictors of Left Ventricular Dysfunction after Aortic Valve Surgery in Children with Chronic Aortic Regurgitation CONGENITAL HEART DISEASE Tierney, E. S., Gal, D., Gauvreau, K., Zhou, J., Soluk, Y., McElhinney, D. B., Colan, S. D., Geva, T. 2013; 8 (4): 308-315

  Abstract

  OBJECTIVE: Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation. METHODS: Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2. RESULTS: Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve. CONCLUSION: Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

  View details for DOI 10.1111/chd.12009

  View details for Web of Science ID 000326501000014

• Infective Endocarditis After Transcatheter Pulmonary Valve Replacement Using the Melody Valve: Combined Results of 3 Prospective North American and European Studies CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Benson, L. N., Eicken, A., Kreutzer, J., Padera, R. F., Zahn, E. M. 2013; 6 (3): 292-300

  Abstract

  Transcatheter (percutaneous) pulmonary valve (TPV) replacement has emerged as a viable therapy for right ventricular outflow tract conduit dysfunction. Little is known about the incidence, clinical course, and outcome of infective endocarditis (IE) after TPV implant. We reviewed combined data from 3 ongoing prospective multicenter trials to evaluate the experience with IE among patients undergoing TPV replacement using the Melody valve.Any clinical episode reported by investigators as IE with documented positive blood cultures and fever, regardless of TPV involvement, was considered IE. Cases were classified as TPV-related if there was evidence of vegetations on or new dysfunction of the TPV. The 3 trials included 311 patients followed for 687.1 patient-years (median, 2.5 years). Sixteen patients were diagnosed with IE 50 days to 4.7 years after TPV implant (median, 1.3 years), including 6 who met criteria for TPV-related IE: 3 with vegetations, 2 with TPV dysfunction, and 1 with both. The annualized rate of a first episode of IE was 2.4% per patient-year and of TPV-related IE was 0.88% per patient-year. Freedom from TPV-related IE was 97±1% 4 years after implant. All patients were treated with intravenous antibiotics, 4 had the valve explanted, and 2 received a second TPV. There was 1 sepsis-related death, 1 patient died of sudden hemoptysis, and 2 patients developed recurrent IE.Bacterial endocarditis has occurred in all 3 prospective multicenter studies of the Melody valve in North America and Europe. Most cases did not involve the TPV and responded to antibiotics. More data are necessary to understand risk factors in this population.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.000087

  View details for Web of Science ID 000320759800020

  View details for PubMedID 23735475

• Transcatheter Closure of Post-myocardial Infarction Ventricular Septal Rupture CIRCULATION-CARDIOVASCULAR INTERVENTIONS Assenza, G. E., McElhinney, D. B., Valente, A. M., Pearson, D. D., Volpe, M., Martucci, G., Landzberg, M. J., Lock, J. E. 2013; 6 (1): 59-?

  Abstract

  Ventricular septal rupture (VSR) after acute myocardial infarction (AMI) is a potentially lethal mechanical complication of acute coronary syndromes. Given high surgical mortality, transcatheter closure has emerged as a potential strategy in selected cases. We report our single-center experience with double-umbrella device percutaneous closure of post-AMI VSR.In this single-center, retrospective, cohort study, patients who underwent transcatheter closure of post-AMI VSR between 1988 and 2008 at Boston Children's Hospital were included. Data were analysed according to whether the patients underwent direct percutaneous VSR closure or closure of a residual VSR after a previous surgical approach. Primary outcome was mortality rate at 30 days. Clinical predictors of primary outcome were investigated using univariate logistic regression. Thirty patients were included in the study (mean age, 67±8 years). A total of 40 closure devices were implanted. Major periprocedural complications occurred in 4 (13%) patients. Cardiogenic shock, increasing pulmonary/systemic flow ratio, and the use of the new generation (6-arm) STARFlex device all were associated with higher risk of mortality. The Model for End-Stage Liver Disease Excluding international normalized ratio (MELD-XI) score at the time of VSR closure seemed to be most strongly associated with death (odds ratio, 1.6; confidence interval, 1.1-2.2; P<0.001).Transcatheter closure of post-AMI VSR using CardioSEAL or STARFlex devices is feasible and effective. The MELD-XI score, a marker of multiorgan dysfunction, is a promising risk stratifier in this population of patients. Early closure of post-AMI VSR is advisable before establishment of multiorgan failure.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.972711

  View details for Web of Science ID 000330360900016

  View details for PubMedID 23339839

• Left Ventricular Diastolic Function in Children and Young Adults With Congenital Aortic Valve Disease AMERICAN JOURNAL OF CARDIOLOGY Friedman, K. G., McElhinney, D. B., Rhodes, J., Powell, A. J., Colan, S. D., Lock, J. E., Brown, D. W. 2013; 111 (2): 243-249

  Abstract

  Young patients with congenital aortic valve disease are at risk of left ventricular (LV) diastolic dysfunction (DD). We evaluated LV remodeling and the prevalence of, and risk factors for, DD in patients with aortic stenosis (AS), pure aortic regurgitation (AR), and AS+AR. Patients aged 8 to 39 years with congenital AS (n = 103), AR (n = 36), or AS+AR (n = 107) were identified. Cross-sectional assessment of the LV remodeling pattern and diastolic function was performed. A diastolic function score (DFS; range 0 to 4) was assigned to each patient, with 1 point for an abnormal value in each of 4 categories: mitral inflow (E/A and E-wave deceleration time), tissue Doppler E', E/E', and left atrial volume. Patients with a DFS of ≥2 were compared to those with a DFS <2. Concentric hypertrophy was the most common remodeling pattern in those with AS (51%), mixed/physiologic hypertrophy in those with AS+AR (48%) and eccentric hypertrophy in those with AR (49%) predominated. In the entire cohort, 91 patients (37%) had a DFS of ≥2. Patients with AS or AS+AR had greater DFS than those with pure AR (p <0.001). On multivariate analysis, a greater LV mass z-score and previous aortic valve balloon dilation were associated with a DFS of ≥2. In patients with catheterization data (n = 65), E/E' correlated with LV end-diastolic pressure. Those with a DFS of ≥2 had a greater LV end-diastolic pressure and mean pulmonary artery pressure than those with a DFS <2. In conclusion, DD is common in young patients with AS and AS+AR but not in those with pure AR. A greater LV mass and previous aortic valve dilation were associated with DD.

  View details for DOI 10.1016/j.amjcard.2012.09.026

  View details for Web of Science ID 000313607100016

  View details for PubMedID 23102884

• Bare metal stenting for obstructed small diameter homograft conduits in the right ventricular outflow tract CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Carr, M., Bergersen, L., Marshall, A. C., Keane, J. F., Lock, J. E., Emani, S. M., McElhinney, D. B. 2013; 81 (1): E44-E52

  Abstract

  Our aim was to assess acute hemodynamic changes with stent insertion, outcomes, and factors associated with increased longevity of stented small diameter homograft conduits.Right ventricle-to-pulmonary artery (RV-PA) homograft conduits are commonly used to palliate RV outflow tract obstruction. Bare metal stenting (BMS) and transcatheter pulmonary valve implantation have been shown to relieve obstructed larger diameter conduits and may delay surgical conduit reintervention. Less is known about BMS of small conduits.From 1992 to 2009, BMS was performed to relieve obstruction in 106 homograft conduits that were ≤12 mm at implant. The peak RV-PA gradient fell from 54.3 ± 17.4 mm Hg at baseline to 46.1 ± 15.2 mm Hg after balloon dilation alone and to 25.1 ± 11.4 mm Hg with stenting (all P < 0.001). Higher pre-BMS gradient and RV pressure were the only factors associated with higher post-BMS RV-PA gradient (≥30 mm Hg; both P < 0.001). There were no procedural deaths, two patients required surgical removal of embolized stents. At a median follow-up of 1.6 years, 83 conduits were replaced; freedom from conduit reoperation after BMS was 66% ± 5% at 1 year and 28% ± 5% at 3 years. Factors associated with shorter freedom from reoperation included implanted conduit diameter <10 mm (P = 0.009), higher post-stent RV-PA gradient (P = 0.026), and higher post-stent RV pressure (P < 0.01); only post-stent RV pressure remained significant on multivariable analysis (P < 0.001).BMS was acutely effective for the treatment of obstructed small diameter homograft conduits, with low morbidity. Prolongation of small diameter homograft conduit longevity with BMS may be useful in the lifetime management of conduit dysfunction in this patient population.

  View details for DOI 10.1002/ccd.24369

  View details for Web of Science ID 000312942300007

  View details for PubMedID 22431478

• Obstruction of the Superior Vena Cava After Neonatal Extracorporeal Membrane Oxygenation: Association With Chylothorax and Outcome of Transcatheter Treatment PEDIATRIC CRITICAL CARE MEDICINE Kazanci, S. Y., McElhinney, D. B., Thiagarajan, R., Bergersen, L., Wilson, J. M., Marshall, A. C., Lock, J. E., Mullen, M. P. 2013; 14 (1): 37-43

  Abstract

  Obstruction of the superior vena cava is one of the potential complications of neonatal extracorporeal membrane oxygenation. Chylothorax is a known complication of surgery involving the thoracic cavity in children, and of extracorporeal membrane oxygenation. The aim of this study was to evaluate the association between chylothorax and superior vena cava obstruction after neonatal extracorporeal membrane oxygenation.Twenty-two patients diagnosed with superior vena cava obstruction at ≤ 6 months of age (median 1.8 months) after neonatal extracorporeal membrane oxygenation were compared with a randomly selected cohort of 44 neonatal extracorporeal membrane oxygenation patients without superior vena cava obstruction. Among patients with superior vena cava obstruction, 18 underwent extracorporeal membrane oxygenation for respiratory disease and four for cardiac insufficiency. Chylothorax was more prevalent among patients with superior vena cava obstruction than controls (odds ratio 9.4 [2.2-40], p = .01) and was associated with extension of obstruction into the left innominate vein. Patients with superior vena cava obstruction were supported by extracorporeal membrane oxygenation for a longer duration than controls. Nineteen patients with superior vena cava obstruction (86%) underwent transcatheter balloon angioplasty and/or stent implantation (median 7 days after diagnosis), which decreased the superior vena cava pressure and superior vena cava-to-right atrium pressure gradient and increased the superior vena cava diameter (all p < 0.001). There were no serious procedural adverse events. Six study patients died within 30 days of the diagnosis of superior vena cava obstruction (including three of nine with chylothorax), which did not differ from controls. During a median follow-up of 2.7 yrs, two additional patients died and nine underwent 14 superior vena cava reinterventions.Among neonates treated with extracorporeal membrane oxygenation, superior vena cava obstruction is associated with an increased risk of chylothorax. In neonates with chylothorax after extracorporeal membrane oxygenation, evaluation for superior vena cava obstruction may be warranted. Although mortality is high in this population, transcatheter treatment can relieve superior vena cava obstruction and facilitate symptomatic improvement.

  View details for DOI 10.1097/PCC.0b013e31825b5270

  View details for Web of Science ID 000313352200011

  View details for PubMedID 23295835

• Melody Valve Implant Within Failed Bioprosthetic Valves in the Pulmonary Position A Multicenter Experience CIRCULATION-CARDIOVASCULAR INTERVENTIONS Gillespie, M. J., Rome, J. J., Levi, D. S., Williams, R. J., Rhodes, J. F., Cheatham, J. P., Hellenbrand, W. E., Jones, T. K., Vincent, J. A., Zahn, E. M., McElhinney, D. B. 2012; 5 (6): 862-870

  Abstract

  Transcatheter pulmonary valve implantation using the Melody valve has emerged as an important therapy for the treatment of postoperative right ventricular outflow tract dysfunction. Melody-in-bioprosthetic valves (BPV) is currently considered an off-label indication. We review the combined experience with transcatheter pulmonary valve implantation within BPVs from 8 centers in the United States and discuss technical aspects of the Melody-in-BPV procedure.A total of 104 patients underwent Melody-in-BPV in the pulmonary position at 8 US centers from April 2007 to January 2012. Ten different types of BPVs were intervened on, with Melody valve implantation at the intended site in all patients. Following Melody valve implant, the peak right ventricle-to-pulmonary artery gradient decreased from 38.7 ± 16.3 to 10.9 ± 6.7 mm Hg (P<0.001), and the right ventricular systolic pressure fell from 71.6 ± 21.7 to 46.7 ± 15.9 mm Hg (P<0.001). There was no serious procedural morbidity, and no deaths related to the catheterization or implant. At a median follow-up of 12 months (1-46 months), no patients had more than mild regurgitation, and 4 had a mean right ventricular outflow tract gradient ≥30 mm Hg. During follow-up, there were 2 stent fractures, 3 cases of endocarditis (2 managed with surgical explant), and 2 deaths that were unrelated to the Melody valve.Transcatheter pulmonary valve implantation using the Melody valve within BPVs can be accomplished with a high rate of success, low procedure-related morbidity and mortality, and excellent short-term results. The findings of this preliminary multicenter experience suggest that the Melody valve is an effective transcatheter treatment option for failed BPVs.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.972216

  View details for Web of Science ID 000313576500026

  View details for PubMedID 23212395

• Staged Left Ventricular Recruitment After Single-Ventricle Palliation in Patients With Borderline Left Heart Hypoplasia JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Emani, S. M., McElhinney, D. B., Tworetzky, W., Myers, P. O., Schroeder, B., Zurakowski, D., Pigula, F. A., Marx, G. R., Lock, J. E., del Nido, P. J. 2012; 60 (19): 1966-1974

  Abstract

  The goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP).Management of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV.Patients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group.Mean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum.In these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.

  View details for DOI 10.1016/j.jacc.2012.07.041

  View details for Web of Science ID 000310352600019

  View details for PubMedID 23062531

• Dilation of the Ascending Aorta After Balloon Valvuloplasty for Aortic Stenosis During Infancy and Childhood AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Lacro, R. V., Gauvreau, K., O'Brien, C. M., Kazanci, S. Y., Vogel, M., Emani, S., Brown, D. W. 2012; 110 (5): 702-708

  Abstract

  Dilation of the ascending aorta (AA) is common in patients with a bicuspid aortic valve. The natural history of the aortic root and AA and the risk factors for dilation have not been characterized in patients with congenital aortic stenosis (AS) treated with balloon valvuloplasty during childhood. The present study was performed to determine the prevalence of aortic dilation in patients with congenital AS before and up to 20 years after balloon valvuloplasty performed during childhood. In patients who underwent balloon valvuloplasty for AS at age ≤ 18 years from 1984 to 2005, the aortic diameter measurements before intervention and at 5-year intervals afterward were recorded and the Z scores calculated. Among 156 patients (median age 1.5 years at valvuloplasty), the AA Z scores were significantly larger than normal before intervention (median Z score 1.5) and at all follow-up points (all p <0.001). Using mixed modeling, with time as a categorical variable (before intervention, 5-year window, 10-year window, and so forth), the mean AA Z score was greater at all postvalvuloplasty points than before the intervention, with mean Z score increases of 1.20 at 5 years and 2.11 at 20 years (p <0.001). Moderate or greater aortic regurgitation early after valvuloplasty was associated with greater AA Z scores than mild or less aortic regurgitation, with a progressive difference over time. More significant residual AS after valvuloplasty was associated with lower AA Z scores over time. In conclusion, AA dilation is common in children with congenital AS and continues to progress over many years after balloon valvuloplasty.

  View details for DOI 10.1016/j.amjcard.2012.04.053

  View details for Web of Science ID 000308618200015

  View details for PubMedID 22608951

• Clinical and Stent-Related Outcomes After Transcatheter or Operative Placement of Bare-Metal Stents in the Ventricular Septum or Subvalvar Systemic Outflow Tract CIRCULATION-CARDIOVASCULAR INTERVENTIONS Porras, D., McElhinney, D. B., del Nido, P., Lock, J. E., Meadows, J., Marshall, A. C. 2012; 5 (4): 570-581

  Abstract

  Stenting of the systemic ventricular outflow tract and creation or enlargement of a ventricular septal defect using stents are potential therapeutic options in rare patients with congenital heart disease who develop significant ventricular hypertension due to outflow obstruction. These stents may be at increased risk of fracture because of exposure to cyclic compressive stresses. The objective of this study was to evaluate the safety and efficacy of this intervention and to determine the incidence of stent fracture and other adverse outcomes after placement of intraventricular or interventricular stents for this indication.Between 1992 and 2010, 27 patients underwent bare-metal stent placement in the ventricular septum or subvalvar systemic outflow tract, using 1 of the following 3 delivery approaches: (1) percutaneous (n=18), (2) intraoperative (n=8), and (3) hybrid (n=1). The median ventricular-to-aortic systolic pressure ratio decreased from 1.7 (1.3-2.6) to 1.1 (0.9-1.8) (P<0.001). Survival free from surgical reintervention for outflow obstruction was 92% at 1 year and 66% at 3 years. Stent fracture was diagnosed in 11 patients (41%) a median of 2.6 years (0.03-7.8 years) after stent placement and was always associated with recurrent obstruction. Survival free from diagnosis of stent fracture was 84% at 1 year and 73% at 3 years. Other adverse events included complete heart block (n=2) and increased atrioventricular valve regurgitation requiring surgical intervention (n=2).Transcatheter, intraoperative, or hybrid stenting of the ventricular septum or systemic outflow tract is feasible and effective in the short term. Stent fractures were common during follow-up and associated with recurrent obstruction.

  View details for DOI 10.1161/CIRCINTERVENTIONS.111.967190

  View details for Web of Science ID 000313575600021

  View details for PubMedID 22851527

• Left Ventricular Remodeling and Improvement in Diastolic Function After Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis CIRCULATION-CARDIOVASCULAR INTERVENTIONS Friedman, K. G., McElhinney, D. B., Colan, S. D., Porras, D., Powell, A. J., Lock, J. E., Brown, D. W. 2012; 5 (4): 549-554

  Abstract

  In congenital aortic stenosis, chronic pressure load has detrimental effects on left ventricular (LV) systolic and diastolic function. Reduction in LV pressure load with balloon aortic valvuloplasty (BAVP) may improve diastolic function.Echocardiographic and catheterization data for 25 consecutive patients undergoing BAVP for congenital aortic stenosis were retrospectively analyzed. Median age at BAVP was 11.5 years (3.2-40.1). LV end-diastolic pressure was elevated (≥15 mm Hg) in 72% of patients, with a median of 17 mm Hg (range, 9-24). With BAVP, median aortic stenosis gradient was reduced from 63 mm Hg (range, 44-105) to 30 mm Hg (range, 10-43). Aortic regurgitation increased from trivial (none to mild) to mild (trivial to moderate). Pre-BAVP early diastolic mitral inflow velocity/tissue Doppler early diastolic velocity (E/E´) correlated with LV end-diastolic pressure (r=0.52, P=0.007). On follow-up echocardiography (median, 11 months after BAVP), aortic stenosis gradient was lower (P<0.001) and degree of AR was higher (P=0.01) compared with pre-BAVP echocardiograms. LV end-diastolic volume z-score increased (P=0.02), LV mass was unchanged, and LV mass:volume decreased (P=0.002). Mitral annular and septal E´ (P<0.001) were higher and E/E´ was lower after dilation (10.8 versus 14.2, P<0.001). Lower pre-BAVP E/E´ and lower pre-BAVP LV mass z-score were associated with lower post-BAVP E/E.After BAVP, LV remodeling characterized by an increase in EDV and decrease in LV mass:volume occurs and echocardiographic measures of diastolic function and LV end-diastolic pressure improve in most patients. Risk factors for persistent diastolic dysfunction include higher pre-BAVP LV mass z-score and worse pre-BAVP diastolic function.

  View details for DOI 10.1161/CIRCINTERVENTIONS.112.968958

  View details for Web of Science ID 000313575600018

  View details for PubMedID 22739787

• Left ventricular function and geometry in fetuses with severe tricuspid regurgitation ULTRASOUND IN OBSTETRICS & GYNECOLOGY Ishii, T., Tworetzky, W., Harrild, D. M., Marcus, E. N., McElhinney, D. B. 2012; 40 (1): 55-61

  Abstract

  Neonatal congenital tricuspid valve (TV) dysplasia and/or displacement (Ebstein's malformation) with severe tricuspid regurgitation (TR) is a challenging condition in which outcomes are frequently poor. Little is known about left ventricular (LV) function during the perinatal period in patients with congenital TV disease. The objective of this study was to evaluate LV function in fetuses with congenital TV anomalies associated with significant TR.Serial fetal echocardiograms in 16 fetuses with congenital TV dysplasia and/or displacement (five neonatal survivors and 11 fetal or neonatal deaths) were reviewed. LV stroke volume, LV end-diastolic volume (LVEDV), LV end-diastolic dimension (LVIDd), the LV eccentricity index, thoracic and cardiac areas and the cardiothoracic area ratio (CTAR), the right atrium area index, and LV longitudinal strains were compared according to gestational age and clinical outcome.The gestational age-adjusted LVEDV (Z-score) was lower in late gestation (-1.2 ± 1.2 at last examination ≥ 28 weeks) than earlier in gestation (0.3 ± 1.5 at last examination < 28 weeks) and LV output was lower than reported late-gestation normal values. LV short-axis dimension correlated with LV volume and CTAR. LV mid-septal strain was lower than the normal average of fetal mid-septal strain and correlated with the LV eccentricity index. Among these parameters, only the LV eccentricity index differed between survivors and non-survivors.LV function and anatomy are abnormal in fetuses with severe congenital TV anomalies and may be important contributors to outcome.

  View details for DOI 10.1002/uog.10115

  View details for Web of Science ID 000305822800008

  View details for PubMedID 21997973

• Reliability and Accuracy of Echocardiographic Right Heart Evaluation in the US Melody Valve Investigational Trial JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Brown, D. W., McElhinney, D. B., Araoz, P. A., Zahn, E. M., Vincent, J. A., Cheatham, J. P., Jones, T. K., Hellenbrand, W. E., O'Leary, P. W. 2012; 25 (4): 383-U138

  Abstract

  Appropriate patient selection for transcatheter pulmonary valve (TPV) replacement requires accurate evaluation of right ventricular (RV) performance. The aim of this study was to evaluate the reliability and accuracy of echocardiography for evaluating RV parameters in patients in the five-center Melody TPV trial.Echocardiographic data were compared with cardiac magnetic resonance (CMR) and catheterization; interobserver comparisons were made using site and core laboratory data.Doppler echocardiographic assessments of RV outflow tract obstruction and RV pressure showed excellent interobserver agreement; mean Doppler gradients were correlated most closely with gradients at catheterization (R = 0.66), and Doppler RV pressure estimates were correlated well with catheterization data (R = 0.58). Assessment of pulmonary regurgitation (PR) using a three-point severity scale showed good agreement with CMR-derived PR fraction (86%). The tricuspid annular Z score was highly reproducible but correlated weakly with CMR RV end-diastolic volume (R = 0.21). However, RV apical diastolic area was highly reproducible (R = 0.87) and had an excellent correlation with CMR RV end-diastolic volume (R = 0.78); all patients with indexed RV apical diastolic areas ≥30 cm(2)/m(2) had CMR RV end-diastolic volumes ≥160 mL/m(2). RV function using the fractional area change method showed a fair correlation with CMR RV ejection fraction (R = 0.48).In patients with dysfunctional RV outflow tract conduits, echocardiography provided reproducible, accurate estimates of pressure overload and RV size. Echocardiographic assessment of PR correlated less closely with CMR PR fraction but showed good categorical agreement; assessment of RV function by these methods was suboptimal. Echocardiography alone may be a suitable screening test for some TPV replacement candidates; CMR may be indicated for TPV replacement decisions hinging on assessment of RV function.

  View details for DOI 10.1016/j.echo.2011.12.022

  View details for Web of Science ID 000302131900004

  View details for PubMedID 22265459

• Outcomes After Stent Implantation for the Treatment of Congenital and Postoperative Pulmonary Vein Stenosis in Children CIRCULATION-CARDIOVASCULAR INTERVENTIONS Balasubramanian, S., Marshall, A. C., Gauvreau, K., Peng, L. F., Nugent, A. W., Lock, J. E., McElhinney, D. B. 2012; 5 (1): 109-117

  Abstract

  Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid.We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases.Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

  View details for DOI 10.1161/CIRCINTERVENTIONS.111.964189

  View details for Web of Science ID 000300610900021

  View details for PubMedID 22253356

• Cardiopulmonary exercise function among patients undergoing transcatheter pulmonary valve implantation in the US Melody valve investigational trial AMERICAN HEART JOURNAL Batra, A. S., McElhinney, D. B., Wang, W., Zakheim, R., Garofano, R. P., Daniels, C., Yung, D., Cooper, D. M., Rhodes, J. 2012; 163 (2): 280-287

  Abstract

  We assessed the hypothesis that there is an improvement in clinical and physiologic parameters of cardiopulmonary exercise testing (CPET) after implantation of a transcatheter pulmonary valve (TPV).Transcatheter pulmonary valve provides a new tool for treating conduit stenosis and regurgitation in patients with right ventricle (RV) to pulmonary artery conduit dysfunction.Patients who underwent a TPV placement between January 2007 and January 2010 (N = 150) were investigated with a standardized CPET protocol before and at 6 months after TPV placement. Cardiopulmonary exercise testing was performed on a mechanically braked cycle ergometer with respiratory gas exchange analysis.Six months post TPV, small but statistically significant improvements were observed in the maximum workload (65.0% ± 18.8% to 68.3% ± 20.3% predicted, P < .001) and the ratio of minute ventilation to CO(2) production at the anaerobic threshold (30.8 ± 4.7 to 29.1 ± 4.1, P < .001). There was no significant change in peak oxygen consumption (VO(2)). Patients with pre-TPV hemodynamics consistent with RV dysfunction and patients with a lower pre-TPV peak VO(2) tended to have the greatest improvement in peak VO(2). The correlation between TPV-related improvements in peak VO(2) and baseline clinical variables were weak, however, and these variables could not be used to reliably identify patients likely to have improved peak VO(2) after TPV.In patients with RV to pulmonary artery conduit dysfunction, TPV is associated with modest improvement in exercise capacity and gas exchange efficiency during exercise.

  View details for DOI 10.1016/j.ahj.2011.10.017

  View details for Web of Science ID 000300226600022

  View details for PubMedID 22305848

• Stent Fracture, Valve Dysfunction, and Right Ventricular Outflow Tract Reintervention After Transcatheter Pulmonary Valve Implantation Patient-Related and Procedural Risk Factors in the US Melody Valve Trial CIRCULATION-CARDIOVASCULAR INTERVENTIONS McElhinney, D. B., Cheatham, J. P., Jones, T. K., Lock, J. E., Vincent, J. A., Zahn, E. M., Hellenbrand, W. E. 2011; 4 (6): 602-U130

  Abstract

  Among patients undergoing transcatheter pulmonary valve (TPV) replacement with the Melody valve, risk factors for Melody stent fracture (MSF) and right ventricular outflow tract (RVOT) reintervention have not been well defined.From January 2007 to January 2010, 150 patients (median age, 19 years) underwent TPV implantation in the Melody valve Investigational Device Exemption trial. Existing conduit stents from a prior catheterization were present in 37 patients (25%, fractured in 12); 1 or more new prestents were placed at the TPV implant catheterization in 51 patients. During follow-up (median, 30 months), MSF was diagnosed in 39 patients. Freedom from a diagnosis of MSF was 77±4% at 14 months (after the 1-year evaluation window) and 60±9% at 39 months (3-year window). On multivariable analysis, implant within an existing stent, new prestent, or bioprosthetic valve (combined variable) was associated with longer freedom from MSF (P<0.001), whereas TPV compression (P=0.01) and apposition to the anterior chest wall (P=0.02) were associated with shorter freedom from MSF. Freedom from RVOT reintervention was 86±4% at 27 months. Among patients with a MSF, freedom from RVOT reintervention after MSF diagnosis was 49±10% at 2 years. Factors associated with reintervention were similar to those for MSF.MSF was common after TPV implant in this multicenter experience and was more likely in patients with severely obstructed RVOT conduits and when the TPV was directly behind the anterior chest wall and/or clearly compressed. A TPV implant site protected by a prestent or bioprosthetic valve was associated with lower risk of MSF and reintervention.

  View details for DOI 10.1161/CIRCINTERVENTIONS.111.965616

  View details for Web of Science ID 000300549500012

  View details for PubMedID 22075927

• Short-Term Performance of the Transcatheter Melody Valve in High-Pressure Hemodynamic Environments in the Pulmonary and Systemic Circulations CIRCULATION-CARDIOVASCULAR INTERVENTIONS Hasan, B. S., McElhinney, D. B., Brown, D. W., Cheatham, J. P., Vincent, J. A., Hellenbrand, W. E., Jones, T. K., Zahn, E. M., Lock, J. E. 2011; 4 (6): 615-620

  Abstract

  The Melody valve is approved for percutaneous pulmonary valve replacement in dysfunctional right ventricular outflow tracts. The function of this valve when subjected to high-pressure loads in humans is unknown. The aim of this study was to describe the immediate and short-term results of Melody valves implanted in a high-pressure environment.Definitions of a high-pressure system were established for Melody valves implanted in the systemic (ie, aortic or mitral position) and pulmonary (ie, right ventricular outflow tract conduit or tricuspid valve annulus) circulations. Implants in these environments were ascertained from databases of the 5 centers that participated in the US Investigational Device Exemption trial. Thirty implants met the inclusion criteria: 23 pulmonary circulation implants (all in the pulmonary position) systemic circulation implants (5 in the native aortic position, 1 in a left ventricle-to-descending aorta conduit, and 1 in the mitral annulus). All pulmonary circulation implants were performed percutaneously in the catheterization laboratory. A hybrid approach (surgical exposure for transcatheter implant) was used for 4 of the aortic implants. There were no procedure-related deaths. Three patients died of nonprocedure- and nonvalve-related causes. At 1 year, freedom from moderate to severe regurgitation was 100%, and freedom from mild regurgitation was 90%. Freedom from moderate to severe stenosis was 86% at 1 year.Short-term performance of the Melody valve in high-pressure environments is encouraging, with good valve function in all patients.

  View details for DOI 10.1161/CIRCINTERVENTIONS.111.963389

  View details for Web of Science ID 000300549500013

  View details for PubMedID 22075926

• The evolving role of intraoperative balloon pulmonary valvuloplasty in valve-sparing repair of tetralogy of Fallot JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Robinson, J. D., Rathod, R. H., Brown, D. W., del Nido, P. J., Lock, J. E., McElhinney, D. B., Bacha, E. A., Marshall, A. C. 2011; 142 (6): 1367-1373

  Abstract

  The late morbidity of pulmonary regurgitation has intensified the interest in valve-sparing repair of tetralogy of Fallot. This study reviewed a single institution's experience with valve-sparing repair and investigated the role of intraoperative balloon valvuloplasty.A retrospective chart review identified 238 patients who underwent complete primary repair of tetralogy of Fallot at less than 180 days of age. Patients were divided into 4 groups on the basis of the type of right ventricular outflow tract repair: transannular patch (n = 111), commissurotomy or standard rigid dilation (n = 71), intraoperative balloon pulmonary valvuloplasty (n = 32), or no valvar intervention (n = 24).Baseline demographic and anatomic factors differed among the 4 procedural groups with substantial overlap. Among 142 patients with pulmonary valve hypoplasia (z score, -2 to -4), 37% had valve-sparing repair. These patients had significant annular growth over time: z score increased 0.67 and 1.00 per year in the intraoperative balloon valvuloplasty (P < .001) and traditional valve-sparing (P < .001) groups, respectively. Rates of valve growth did not differ across groups, but z scores were 0.58 lower for the balloon valvuloplasty group across all time points (P = .001). Freedom from reintervention and surgery was shorter for the balloon valvuloplasty group than for the other groups (P < .001).Patients with tetralogy of Fallot and pulmonary valve hypoplasia who undergo valve-sparing repair with intraoperative balloon valvuloplasty have significant longitudinal annular growth, with normalization of annular size over time. Despite application in patients with more hypoplastic valves, balloon valvuloplasty resulted in similar valve growth and pulmonary regurgitation as traditional methods, but higher rates of reintervention. Although the precise role of this technique needs further refinement, it is likely to be most useful in patients with moderate pulmonary stenosis and moderate pulmonary valve dysplasia.

  View details for DOI 10.1016/j.jtcvs.2011.02.047

  View details for Web of Science ID 000297214200011

  View details for PubMedID 21703641

• Catheterization for Congenital Heart Disease Adjustment for Risk Method (CHARM) JACC-CARDIOVASCULAR INTERVENTIONS Bergersen, L., Gauvreau, K., Foerster, S. R., Marshall, A. C., McElhinney, D. B., Beekman, R. H., Hirsch, R., Kreutzer, J., Balzer, D., Vincent, J., Hellenbrand, W. E., Holzer, R., Cheatham, J. P., Moore, J. W., Burch, G., Armsby, L., Lock, J. E., Jenkins, K. J. 2011; 4 (9): 1037-1046

  Abstract

  This study sought to develop a method to adjust for case mix complexity in catheterization for congenital heart disease to allow equitable comparisons of adverse event (AE) rates.The C3PO (Congenital Cardiac Catheterization Project on Outcomes) has been prospectively collecting data using a Web-based data entry tool on all catheterization cases at 8 pediatric institutions since 2007.A multivariable logistic regression model with high-severity AE outcome was built using a random sample of 75% of cases in the multicenter cohort; the models were assessed in the remaining 25%. Model discrimination was assessed by the C-statistic and calibration with Hosmer-Lemeshow test. The final models were used to calculate standardized AE ratios.Between August 2007 and December 2009, 9,362 cases were recorded at 8 pediatric institutions of which high-severity events occurred in 454 cases (5%). Assessment of empirical data yielded 4 independent indicators of hemodynamic vulnerability. Final multivariable models included procedure type risk category (odds ratios [OR] for category: 2 = 2.4, 3 = 4.9, 4 = 7.6, all p < 0.001), number of hemodynamic indicators (OR for 1 indicator = 1.5, ≥2 = 1.8, p = 0.005 and p < 0.001), and age <1 year (OR: 1.3, p = 0.04), C-statistic 0.737, and Hosmer-Lemeshow test p = 0.74. Models performed well in the validation dataset, C-statistic 0.734. Institutional event rates ranged from 1.91% to 7.37% and standardized AE ratios ranged from 0.61 to 1.41.Using CHARM (Catheterization for Congenital Heart Disease Adjustment for Risk Method) to adjust for case mix complexity should allow comparisons of AE among institutions performing catheterization for congenital heart disease.

  View details for DOI 10.1016/j.jcin.2011.05.021

  View details for Web of Science ID 000295602800015

  View details for PubMedID 21939947

• Echocardiographic Evaluation Before Bidirectional Glenn Operation in Functional Single-Ventricle Heart Disease Comparison to Catheter Angiography CIRCULATION-CARDIOVASCULAR IMAGING Stern, K. W., McElhinney, D. B., Gauvreau, K., Geva, T., Brown, D. W. 2011; 4 (5): 498-505

  Abstract

  Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy.Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%). The left PA was imaged by echocardiography in 83 patients (64%), with 4 of 21 stenoses (19%) diagnosed by catheterization identified; similarly, the right PA was imaged in 81 (62%), and 3 of 17 stenoses (18%) were identified. The distal aortic arch was visualized in 104 (80%), with successful identification of 21 of 27 (78%) of coarctations diagnosed by catheterization. Complete vascular echocardiography (visualization of PAs and aortic arch) occurred in 43% and was not obtained more frequently with sedation.In a large cohort of patients presenting for BDG, evaluation by echocardiography frequently failed to image the PAs and missed the majority of PA stenoses. Sedation did not appear to improve the performance of echocardiography for evaluation of the PAs. Echocardiography cannot be relied on as the sole investigation before BDG.

  View details for DOI 10.1161/CIRCIMAGING.110.963280

  View details for Web of Science ID 000295030600008

  View details for PubMedID 21730025

• Factors Associated With Subsequent Arch Reintervention After Initial Balloon Aortoplasty in Patients With Norwood Procedure and Arch Obstruction JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Porras, D., Brown, D. W., Marshall, A. C., del Nido, P., Bacha, E. A., McElhinney, D. B. 2011; 58 (8): 868-876

  Abstract

  The aim of this study was to determine whether there are identifiable factors associated with increased risk for aortic arch reintervention in patients who have undergone balloon aortoplasty (BD) for aortic arch obstruction (COA) after the Norwood procedure (NP).BD has been shown to be an effective acute therapy for COA after the NP. However, recurrent obstruction requiring repeat intervention is not uncommon.All patients who underwent BD as the initial intervention for COA after the NP from to January 1993 to May 2009 were retrospectively analyzed (n = 116).The median age at initial BD was 4.5 months. The median follow-up period was 3.4 years. Procedures were considered acutely successful in 92% of patients, with a median gradient reduction overall from 24 to 3 mm Hg (p < 0.0001) and a COA diameter increase of 52% (p < 0.0001). By Kaplan-Meier analysis, freedom from reintervention was 69% at 1 year and 58% at 5 years, and freedom from reoperation was 82% at 1 year and 79% at 5 years. By Cox regression analysis, proximal arch obstruction, age <3 months at BD, moderate or greater ventricular dysfunction, moderate or greater atrioventricular valve regurgitation on pre-catheterization echocardiography, and higher post-BD coarctation gradient were associated with shorter freedom from reoperation.Despite a high acute success rate, a significant proportion of patients treated with BD for post-NP COA underwent reintervention during follow-up. The risk for arch reintervention is highest in patients with proximal arch obstruction, those age <3 months at the time of BD, and those with less successful acute results.

  View details for DOI 10.1016/j.jacc.2010.12.050

  View details for Web of Science ID 000293729600014

  View details for PubMedID 21835324

• Postnatal Left Ventricular Diastolic Function After Fetal Aortic Valvuloplasty AMERICAN JOURNAL OF CARDIOLOGY Friedman, K. G., Margossian, R., Graham, D. A., Harrild, D. M., Emani, S. M., Wilkins-Haug, L. E., McElhinney, D. B., Tworetzky, W. 2011; 108 (4): 556-560

  Abstract

  Fetal aortic balloon valvuloplasty (FAV) has shown promise in altering in utero progression of aortic stenosis to hypoplastic left heart syndrome. In patients who achieve a biventricular circulation after FAV, left ventricular (LV) compliance may be impaired. Echocardiographic indexes of diastolic function were compared between patients with biventricular circulation after FAV, congenital aortic stenosis (AS), and age-matched controls. In the neonatal period, patients with FAV had similar LV, aortic, and mitral valve dimensions but more evidence of endocardial fibroelastosis than patients with AS. Patients with FAV underwent more postnatal cardiac interventions than patients with AS (p = 0.007). Mitral annular early diastolic tissue velocity (E') was lower in patients with FAV and those with AS and controls in the neonatal period and over follow-up (p <0.001). Septal E' was similar among all 3 groups in the neonatal period. In follow-up patients, with FAV had lower septal E' than patients with AS or controls (p <0.001). Early mitral inflow velocity/E' was higher in patients with FAV as neonates and at follow-up (p <0.001). Mitral inflow pulse-wave Doppler-derived indexes of diastolic function were similar between groups. In conclusion, echocardiographic evidence of LV diastolic dysfunction is common in patients with biventricular circulation after FAV and persists in short-term follow-up. LV diastolic dysfunction in this unique population may have important implications on long-term risk of left atrial and subsequent pulmonary hypertension.

  View details for DOI 10.1016/j.amjcard.2011.03.085

  View details for Web of Science ID 000294040700013

  View details for PubMedID 21624551

• Percutaneous Tricuspid Valve Replacement in Congenital and Acquired Heart Disease JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Roberts, P. A., Boudjemline, Y., Cheatham, J. P., Eicken, A., Ewert, P., McElhinney, D. B., Hill, S. L., Berger, F., Khan, D., Schranz, D., Hess, J., Ezekowitz, M. D., Celermajer, D., Zahn, E. 2011; 58 (2): 117-122

  Abstract

  This study sought to describe the first human series of percutaneous tricuspid valve replacements in patients with congenital or acquired tricuspid valve (TV) disease.Percutaneous transcatheter heart valve replacement of the ventriculoarterial (aortic, pulmonary) valves is established. Although there are isolated reports of transcatheter atrioventricular heart valve replacement (hybrid and percutaneous), this procedure has been less frequently described; we are aware of no series describing this procedure for TV disease.We approached institutions with significant experience with the Melody percutaneous pulmonary valve (Medtronic, Inc., Minneapolis, Minnesota) to collect data where this valve had been implanted in the tricuspid position. Clinical and procedural data were gathered for 15 patients. Indications for intervention included severe hemodynamic compromise and perceived high surgical risk; all had prior TV surgery and significant stenosis and/or regurgitation of a bioprosthetic TV or a right atrium-to-right ventricle conduit.Procedural success was achieved in all 15 patients. In patients with predominantly stenosis, mean tricuspid gradient was reduced from 12.9 to 3.9 mm Hg (p < 0.01). In all patients, tricuspid regurgitation was reduced to mild or none. New York Heart Association functional class improved in 12 patients. The only major procedural complication was of third-degree heart block requiring pacemaker insertion in 1 patient. One patient developed endocarditis 2 months after implant, and 1 patient with pre-procedural multiorgan failure did not improve and died 20 days after the procedure. The remaining patients have well-functioning Melody valves in the TV position a median of 4 months after implantation.In selected cases, patients with prior TV surgery may be candidates for percutaneous TV replacement.

  View details for DOI 10.1016/j.jacc.2011.01.044

  View details for Web of Science ID 000292189300003

  View details for PubMedID 21718905

• Early Reintervention on the Pulmonary Arteries and Right Ventricular Outflow Tract After Neonatal or Early Infant Repair of Truncus Arteriosus Using Homograft Conduits AMERICAN JOURNAL OF CARDIOLOGY Lund, A. M., Vogel, M., Marshall, A. C., Emani, S. M., Pigula, F. A., Tworetzky, W., McElhinney, D. B. 2011; 108 (1): 106-113

  Abstract

  Patients with truncus arteriosus often require pulmonary arterial (PA) and/or right ventricular outflow tract (RVOT) reintervention within the first year of repair. However, little is known about the risk factors for early reintervention on the PAs or RVOT in this population. The objective of the present retrospective cohort study was to determine the risk factors for early PA or RVOT reintervention after repair of truncus arteriosus in neonates and young infants. Of 156 patients ascertained (median age at repair 14 days; 143 early survivors), reinterventions on the RVOT and/or PAs were performed in 109. The first reintervention was catheter therapy in 73 patients (conduit dilation/stenting in 29, PA dilation/stenting in 31, both in 13) and conduit reoperation in 36 patients. The freedom from any RVOT or PA reintervention was 68 ± 4% at 1 year and 48 ± 5% at 2 years. The factors associated with early reintervention (shorter 1-year freedom from reintervention) on univariate analysis were repair quartile, neonatal repair, smaller weight at repair, and smaller implanted conduit size. On multivariable analysis, only smaller conduit size remained significant (multivariable hazard ratio 0.66/mm, range 0.53 to 0.83; p <0.001). The freedom from conduit reoperation was 92 ± 3% at 1 year and 76 ± 4% at 2 years. Overall, the left and right PA sizes were modestly larger than normal by the 1-sample t test, and PA Z scores and the PA area index were not associated with the risk of reintervention. Early reintervention for PA and/or RVOT conduit obstruction is common after neonatal and early infant repair of truncus arteriosus using homograft conduits. A smaller conduit size was associated with early RVOT/PA reintervention. The branch PA size was normal before surgery, suggesting that the PA stenosis in these patients resulted from factors other than intrinsic stenosis or hypoplasia.

  View details for DOI 10.1016/j.amjcard.2011.03.006

  View details for Web of Science ID 000292785600018

  View details for PubMedID 21530938

• Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY McElhinney, D. B., Marx, G. R., Marshall, A. C., Mayer, J. E., del Nido, P. J. 2011; 141 (6): 1362-U57

  Abstract

  Pulmonary arteriovenous malformations are an important but uncommon complication of cavopulmonary connection, particularly in patients with heterotaxy. Absence of hepatic venous effluent in pulmonary arterial blood seems to be a predisposing factor. Pulmonary arteriovenous malformations are most common after superior cavopulmonary anastomosis, but may develop, progress, or persist in 1 lung after Fontan completion if hepatic venous blood streams completely or primarily to the contralateral lung.Among 53 patients with heterotaxy and inferior vena cava interruption who underwent a modified Fontan procedure from 1985 to 2005, 8 had unilateral streaming of hepatic venous flow and clinically significant pulmonary arteriovenous malformations after hepatic venous inclusion and underwent reconfiguration of the cavopulmonary pathway. In all 8 patients, the hepatic vein-pulmonary artery pathway was contralateral to and offset from the pulmonary artery anastomosis of the single or dominant superior vena cava. Pathway reconfiguration included pulmonary arterial stenting (n = 2), revision of the superior vena cava-pulmonary artery connection (n = 1), construction of a branched hepatic vein-pulmonary artery conduit (n = 2), and surgical or transcatheter construction of a direct hepatic vein-azygous vein pathway (n = 5).Hepatic vein-azygous vein connection led to improvement in 4 of 5 patients; other approaches typically did not lead to improvement.Resolution of hypoxemia after cavopulmonary pathway reconfiguration in patients with unilateral pulmonary arteriovenous malformations and hepatic venous flow-streaming after Fontan completion supports the importance of hepatic venous effluent in the pathogenesis of pulmonary arteriovenous malformations and the practice of cavopulmonary pathway revision in such patients. Completion or reconfiguration of the Fontan circulation with direct hepatic vein-azygous vein connection may provide the most reliable mixing and bilateral distribution of hepatic venous blood in this population of patients.

  View details for DOI 10.1016/j.jtcvs.2010.08.088

  View details for Web of Science ID 000290710900011

  View details for PubMedID 21146835

• Assessment of Left Ventricular Endocardial Fibroelastosis in Fetuses With Aortic Stenosis and Evolving Hypoplastic Left Heart Syndrome AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Vogel, M., Benson, C. B., Marshall, A. C., Wilkins-Haug, L. E., Silva, V., Tworetzky, W. 2010; 106 (12): 1792-1797

  Abstract

  Systematic evaluation of left ventricular (LV) endocardial fibroelastosis (EFE) in the fetus has not been reported. The role of EFE in the pre- and postnatal evolution of hypoplastic left heart disease, and the implications of EFE for outcomes after prenatal intervention for fetal aortic stenosis with evolving hypoplastic left heart syndrome have also not been determined. A 4-point grading system (0-3) was devised for the assessment of fetal LV echogenicity, which was presumed to be due to EFE. Two reviewers independently graded EFE on the preintervention echocardiograms of fetuses treated with in utero aortic valvuloplasty for evolving hypoplastic left heart syndrome from 2000 to 2008. Intra- and interobserver reproducibility was determined for the EFE grade and characterization of related echocardiographic features. The relations among EFE severity, other left heart anatomic and physiologic variables, and postintervention outcomes were analyzed. The assessment and grading of EFE was possible for both observers in all 74 fetuses studied. By consensus, the EFE severity was grade 1 in 31 patients, grade 2 in 32, and grade 3 in 11. Fetuses with mild (grade 1) EFE had significantly greater maximum instantaneous aortic stenosis gradients (e.g., higher LV pressures) and less globular LV geometry than patients with grade 2 or 3 EFE on preintervention echocardiogram. The severity of EFE was not associated with the size of the aortic valve or LV. From preintervention to late gestation, the time-indexed change in LV end-diastolic volume was significantly greater in fetuses with grade 1 EFE than those with more severe EFE. Incorporation of EFE severity into our previously published threshold score improved the sensitivity and positive predictive value for the postnatal biventricular outcomes. In conclusion, echocardiographic grading of EFE is possible, with reasonable intra- and interobserver reliability in midgestation fetuses with evolving hypoplastic left heart syndrome. EFE severity corresponded to some indexes of left heart size, geometry, and function and with the probability of a biventricular outcome postnatally. Additional experience and external validation of the EFE grading scoring system are necessary.

  View details for DOI 10.1016/j.amjcard.2010.08.022

  View details for Web of Science ID 000285735000020

  View details for PubMedID 21126622

• Sudden Unexpected Death After Balloon Valvuloplasty for Congenital Aortic Stenosis JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Brown, D. W., Dipilato, A. E., Chong, E. C., Gauvreau, K., McElhinney, D. B., Colan, S. D., Lock, J. E. 2010; 56 (23): 1939-1946

  Abstract

  The aims of this study were to determine the incidence and risk factors of sudden unexpected death (SUD) after balloon aortic valvuloplasty (BAVP) for congenital aortic stenosis (AS) and to assess the effect of exercise restriction.Exercise restriction is recommended for some patients with congenital AS because of a perceived increased risk for SUD. Little is known about the incidence of SUD in those with treated AS or the efficacy of exercise restriction in preventing SUD.A review was conducted of 528 patients who underwent BAVP for congenital AS at Children's Hospital Boston from 1984 to 2008. Exercise restriction status was ascertained for those ≥4 years of age, censored at aortic valve replacement or transplantation.Median subsequent follow-up was 12.0 years (range 0 to 24.8 years), for a total of 6,344 patient-years of follow-up. There were 63 deaths, with SUD in 6 patients, 5 of which occurred at ≤18 months of age. For patients ≥4 years of age at most recent follow-up with no histories of pulmonary hypertension (n = 422), median follow-up after BAVP was 14.6 years, for 6,019 patient-years of follow-up. Exercise restriction was prescribed in 183 patients (43%; 2,541 patient-years) and no restriction in 220 (52%; 2,691 patient-years); there were insufficient data in 19 patients. There were 17 deaths in this cohort of 422 patients, with 1 SUD (the patient, who was exercise restricted, died during sleep), for an incidence of 0.18/1,000 patient-years (95% confidence interval: 0.01 of 1,000 to 1.01 of 1,000).SUD is extremely rare after BAVP for congenital AS. No beneficial effect of the recommendation for exercise restriction was observed in this longitudinal cohort with 6,000 patient-years of follow-up.

  View details for DOI 10.1016/j.jacc.2010.06.048

  View details for Web of Science ID 000284571800008

  View details for PubMedID 21109118

• What is the Clinical Utility of Routine Cardiac Catheterization Before a Fontan Operation? PEDIATRIC CARDIOLOGY Banka, P., McElhinney, D. B., Bacha, E. A., Mayer, J. E., Gauvreau, K., Geva, T., Brown, D. W. 2010; 31 (7): 977-985

  Abstract

  Patients with single-ventricle circulation presenting for Fontan completion routinely undergo cardiac catheterization despite ongoing debate concerning its additive value. Increasing interest in noninvasive preoperative evaluation alone led the authors to analyze the utility of routine pre-Fontan catheterization and to determine whether a subset of patients could avoid this invasive procedure. Patients younger than 5 years referred for pre-Fontan evaluation were retrospectively reviewed. Medical records and catheter angiograms were examined, and catheterizations were categorized as "additive" based on predetermined criteria. Associations between precatheterization variables, catheterization findings, and short-term postoperative outcomes were evaluated. Cardiac catheterization was clinically nonadditive for 89 of 175 patients undergoing pre-Fontan evaluation (51%). There were no robust precatheterization predictors of a nonadditive catheterization. Echocardiography did not fully demonstrate the relevant anatomy of 115 patients (66%), most frequently due to inadequate visualization of the pulmonary arteries, and 22 patients had additive catheterizations due to new diagnostic findings alone. Interventions at catheterization were frequent and deemed "important" for 64 patients (37%). Catheterization hemodynamic data were not associated with early postoperative outcomes. Minor catheterization complications occurred for 51 patients (29%) and major complications for 4 patients (2%). Although at least 50% of the patients presenting for Fontan completion may be able to avoid routine catheterization safely, an echocardiography-based imaging strategy alone is insufficient to allow proper identification of those who could be evaluated noninvasively. A more comprehensive imaging strategy not based solely on echocardiography should be considered.

  View details for DOI 10.1007/s00246-010-9736-3

  View details for Web of Science ID 000282424800006

  View details for PubMedID 20503042

• Short- and Medium-Term Outcomes After Transcatheter Pulmonary Valve Placement in the Expanded Multicenter US Melody Valve Trial CIRCULATION McElhinney, D. B., Hellenbrand, W. E., Zahn, E. M., Jones, T. K., Cheatham, J. P., Lock, J. E., Vincent, J. A. 2010; 122 (5): 507-U132

  Abstract

  Transcatheter pulmonary valve placement is an emerging therapy for pulmonary regurgitation and right ventricular outflow tract obstruction in selected patients. The Melody valve was recently approved in the United States for placement in dysfunctional right ventricular outflow tract conduits.From January 2007 to August 2009, 136 patients (median age, 19 years) underwent catheterization for intended Melody valve implantation at 5 centers. Implantation was attempted in 124 patients; in the other 12, transcatheter pulmonary valve placement was not attempted because of the risk of coronary artery compression (n=6) or other clinical or protocol contraindications. There was 1 death from intracranial hemorrhage after coronary artery dissection, and 1 valve was explanted after conduit rupture. The median peak right ventricular outflow tract gradient was 37 mm Hg before implantation and 12 mm Hg immediately after implantation. Before implantation, pulmonary regurgitation was moderate or severe in 92 patients (81% with data); no patient had more than mild pulmonary regurgitation early after implantation or during follow-up (>or=1 year in 65 patients). Freedom from diagnosis of stent fracture was 77.8+/-4.3% at 14 months. Freedom from Melody valve dysfunction or reintervention was 93.5+/-2.4% at 1 year. A higher right ventricular outflow tract gradient at discharge (P=0.003) and younger age (P=0.01) were associated with shorter freedom from dysfunction.In this updated report from the multicenter US Melody valve trial, we demonstrated an ongoing high rate of procedural success and encouraging short-term valve function. All reinterventions in this series were for right ventricular outflow tract obstruction, highlighting the importance of patient selection, adequate relief of obstruction, and measures to prevent and manage stent fracture. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00740870.

  View details for DOI 10.1161/CIRCULATIONAHA.109.921692

  View details for Web of Science ID 000280561100010

  View details for PubMedID 20644013

• To Close or Not to Close: The Very Small Patent Ductus Arteriosus CONGENITAL HEART DISEASE Fortescue, E. B., Lock, J. E., Galvin, T., McElhinney, D. B. 2010; 5 (4): 354-365

  Abstract

  Patent ductus arteriosus (PDA) accounts for approximately 10% of all congenital heart diseases, with an incidence of at least 2-4 per 1000 term births. Closure of the large, hemodynamically significant PDA is established as the standard of care, and can be performed safely and effectively using either surgical or transcatheter methods. The appropriate management of the very small, hemodynamically insignificant PDA is less clear. Routine closure of such defects has been advocated to eliminate or reduce the risk of infective endocarditis (IE). However, the risk of IE in patients with a small PDA appears to be extremely low, and IE is treatable. Although closure of the small PDA is generally safe and technically successful, it is unknown whether this treatment truly improves the risk:benefit balance compared with observation. In this article, we review the published literature on the natural history and treatment outcomes in individuals with a PDA, the epidemiology and outcomes of IE, particularly in association with PDA, and the rationale and evidence for closure of the very small PDA.

  View details for DOI 10.1111/j.1747-0803.2010.00435.x

  View details for Web of Science ID 000289419800005

  View details for PubMedID 20653702

• Comparison of Conventional and Cutting Balloon Angioplasty for Congenital and Postoperative Pulmonary Vein Stenosis in Infants and Young Children CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Peng, L. F., Lock, J. E., Nugent, A. W., Jenkins, K. J., McElhinney, D. B. 2010; 75 (7): 1084-1090

  Abstract

  Pulmonary vein stenosis (PVS) is a rare and often lethal condition in children. The optimal treatment for congenital and postoperative PVS is unknown.We compared outcomes of conventional balloon angioplasty performed for PVS from 1999 to 2003 against cutting balloon angioplasty performed from 2004 to 2007. A total of 100 previously undilated pulmonary veins in 54 patients were studied: 48 veins dilated with conventional balloons and 52 with cutting balloons. Acute results included significantly reduced gradients and increased lumen diameters with both treatments. Acutely, cutting balloon angioplasty and conventional angioplasty yielded similar relative reduction of the PVS gradient (median 78% vs. 63%, P = 0.08) and increase in lumen diameter (median 77% vs. 59%, P = 0.07). There was one procedural death of a critically ill infant, and four cardiac arrests, but no adverse events necessitating surgical intervention. Survival free from reintervention was poor in both groups, and shorter in the cutting balloon group (73% at 1 month, 11% at 6 months, and 4% at 1 year) than in the conventional angioplasty group (77% at 1 month, 35% at 6 months, and 23% at 1 year; P = 0.01).Both conventional and cutting balloon angioplasty were effective at decreasing gradient and increasing lumen size acutely in patients with congenital and postoperative PVS, but reintervention was common with both treatments. Both methods of angioplasty provided limited benefit, and neither was curative for this complex disease.

  View details for DOI 10.1002/ccd.22405

  View details for Web of Science ID 000278503900022

  View details for PubMedID 20146341

• Current Status of Fetal Cardiac Intervention CIRCULATION McElhinney, D. B., Tworetzky, W., Lock, J. E. 2010; 121 (10): 1256-1263

  View details for DOI 10.1161/CIRCULATIONAHA.109.870246

  View details for Web of Science ID 000275604400012

  View details for PubMedID 20231543

• Long-Term Pulmonary Regurgitation Following Balloon Valvuloplasty for Pulmonary Stenosis Risk Factors and Relationship to Exercise Capacity and Ventricular Volume and Function JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Harrild, D. M., Powell, A. J., Trang, T. X., Geva, T., Lock, J. E., Rhodes, J., McElhinney, D. B. 2010; 55 (10): 1041-1047

  Abstract

  This study sought to examine the prevalence and predictors of pulmonary regurgitation (PR) following balloon dilation (BD) for pulmonary stenosis (PS) and to investigate its impact on ventricular volume and function, and exercise tolerance.Balloon pulmonary valvuloplasty relieves PS but can cause late PR. The sequelae of isolated PR are not well understood.Patients were at least 7 years of age and 5 years removed from BD, and had no other form of congenital heart disease or significant residual PS. Cardiac magnetic resonance imaging and exercise testing were performed prospectively to quantify PR fraction, ventricular volumes and function, and exercise capacity.Forty-one patients underwent testing a median of 13.1 years after BD. The median PR fraction was 10%; 14 patients (34%) had PR fraction >15%; 7 (17%) had PR >30%. PR fraction was associated with age at dilation (ln-transformed, R = -0.47, p = 0.002) and balloon:annulus ratio (R = 0.57, p < 0.001). The mean right ventricular (RV) end-diastolic volume z-score was 1.8 +/- 1.9; RV dilation (z-score > or =2) was present in 14/35 patients (40%). PR fraction correlated closely with indexed RV end-diastolic volume (R = 0.79, p < 0.001) and modestly with RV ejection fraction (R = 0.50, p < 0.001). Overall, peak oxygen consumption (Vo(2)) (% predicted) was below average (92 +/- 17%, p = 0.006). Patients with PR fraction >15% had significantly lower peak Vo(2) than those with less PR (85 +/- 17% vs. 96 +/- 16%, p = 0.03).Mild PR and RV dilation are common in the long term following BD. A PR fraction >15% is associated with lower peak Vo(2), suggesting that isolated PR and consequent RV dilation are related to impaired exercise cardiopulmonary function.

  View details for DOI 10.1016/j.jacc.2010.01.016

  View details for Web of Science ID 000275143200013

  View details for PubMedID 20202522

• Significance and outcome of left heart hypoplasia in fetal congenital diaphragmatic hernia ULTRASOUND IN OBSTETRICS & GYNECOLOGY Vogel, M., McElhinney, D. B., Marcus, E., Morash, D., Jennings, R. W., Tworetzky, W. 2010; 35 (3): 310-317

  Abstract

  In patients with a left-sided congenital diaphragmatic hernia (CDH), the left ventricle (LV) is often compressed and smaller than normal. The objective of this study was to investigate whether small left heart dimensions prenatally normalize after birth in patients with CDH, or whether prenatal indices of left heart size and flow predict postnatal outcome.Clinical and echocardiographic data were reviewed for patients diagnosed with left-sided CDH prenatally. Cardiac dimensions and flows were compared with normative data. Among liveborn patients, pre- and postnatal Z-scores of left heart structures were compared, and associations between prenatal indices and outcome were assessed.Of 125 patients diagnosed prenatally with CDH, 111 had a left-sided defect. Of these, 85 were liveborn, including 20 with congenital heart disease. Gestational age-adjusted dimensions of fetal left heart structures, including aortic valve diameter, mitral valve (MV) diameter, LV long-axis, LV short-axis and LV volume, were all smaller than normal (P < 0.001). On average, the LV contributed 33 +/- 8% of combined ventricular output, lower than the normal 40-50%. Z-scores of left heart structures increased from the prenatal echocardiogram to the postnatal study, with average changes ranging from 0.56 +/- 1.68 (aortic valve) to 1.39 +/- 1.85 (LV volume). Among liveborn patients, there was no association between prenatal left heart Z-scores and postnatal survival.Hypoplasia of and reduced flow through the left heart are common among fetuses with CDH. After birth and CDH repair, left heart dimensions generally normalize, with adequate size to support a biventricular circulation, even when there is very low flow through the left heart in mid- and late-gestation.

  View details for DOI 10.1002/uog.7497

  View details for Web of Science ID 000276134800011

  View details for PubMedID 20143332

• Fetal Diagnosis of Interrupted Aortic Arch AMERICAN JOURNAL OF CARDIOLOGY Vogel, M., Vernon, M. M., McElhinney, D. B., Brown, D. W., Colan, S. D., Tworetzky, W. 2010; 105 (5): 727-734

  Abstract

  To determine the frequency of prenatal detection among liveborn patients with an interrupted aortic arch (IAA), the accuracy of prenatal diagnosis, and the anatomic features associated with IAA in the fetus. The prenatal diagnosis of an IAA is challenging. The data on the features and outcomes of fetal IAA are limited. This was a retrospective review of the fetuses and neonates diagnosed with IAA at the Children's Hospital Boston. From 1988 to 2009, 26 fetuses were diagnosed with an IAA. Of these, 21 were live born, and 5 pregnancies were terminated. Of these 21 patients, 18 were confirmed to have an IAA after birth and 3 had severe aortic coarctation. Of the 56 patients diagnosed with an IAA as neonates, 3 had a prenatal echocardiogram that did not include the correct diagnosis. Among the liveborn patients with a postnatally confirmed IAA, 24% were diagnosed prenatally, which increased from 11% during the first 7-year period to 43% more recently. Also, 15% of the prenatally diagnosed patients with IAA had a family history of structural or genetic anomalies. In fetuses with an IAA, echocardiographic Z-scores for the aortic valve and ascending aorta were significantly lower than in normal fetuses, but the left ventricular dimensions were normal. Aortopulmonary diameter ratios were abnormally low. In conclusion, although the identification of IAA on a prenatal echocardiogram can be challenging, a number of anatomic features can facilitate the diagnosis. In particular, a low aortopulmonary diameter ratio in the absence of a ventricular size discrepancy should prompt consideration of this diagnosis. Despite the diagnostic challenges, the frequency of prenatal diagnosis of the IAA is increasing.

  View details for DOI 10.1016/j.amjcard.2009.10.053

  View details for Web of Science ID 000278137100025

  View details for PubMedID 20185024

• Serial Cardiopulmonary Exercise Testing in Patients with Previous Fontan Surgery PEDIATRIC CARDIOLOGY Fernandes, S. M., McElhinney, D. B., Khairy, P., Graham, D. A., Landzberg, M. J., Rhodes, J. 2010; 31 (2): 175-180

  Abstract

  Patients with previous Fontan surgery have reduced peak oxygen consumption (Vo 2), and data regarding progression of exercise intolerance is limited. The purpose of this study was to assess the evolution of exercise tolerance in patients with previous Fontan surgery. We performed a retrospective cohort study of patients with previous Fontan surgery who underwent cardiopulmonary exercise testing between November 2002 and January 2009. Patients were required to have completed at least two tests, with adequate levels of effort, >or-6 months apart. We identified 78 patients (55% male) who had undergone a total of 215 cardiopulmonary exercise tests. Age at initial study was 19.7 +/- 10.2 years; time interval since previous Fontan surgery 13.3 +/- 5.7 years; and time interval between the initial and the most recent exercise test was 3.0 +/- 1.4 years. Morphologic left ventricle was present in 58 (74.4%) patients. At baseline, peak Vo 2 averaged 24.7 +/- 7.0 ml/kg/min (63.7% +/- 15.8% predicted). At most recent study, peak Vo 2 averaged 23.2 +/- 7.2 ml/kg/min, (60.4% +/- 13.9% predicted). Percent predicted peak Vo 2 declined slowly, with a mean rate of decline of 1.25 +/- 0.36 percentage points/y (p < 0.001). Most of the decline occurred in patients < 18 years of age (mean rate of decline 1.78 +/- 0.46 percentage points/y; p = 0.0004). Thereafter, the rate of decline was less marked (mean rate of decline 0.54 +/- 0.57 percentage points/y; p = not significant). Ventricular morphology, type of Fontan procedure, and cardiac medications were not predictive of progressive exercise intolerance. In conclusion, although the exercise function of previous Fontan patients tends to decline during late adolescence, it appears to stabilize during early adulthood.

  View details for DOI 10.1007/s00246-009-9580-5

  View details for Web of Science ID 000274333900001

  View details for PubMedID 19915891

• Reversible Ductus Arteriosus Constriction due to Maternal Indomethacin after Fetal Intervention for Hypoplastic Left Heart Syndrome with Intact/Restrictive Atrial Septum FETAL DIAGNOSIS AND THERAPY Vogel, M., Wilkins-Haug, L. E., McElhinney, D. B., Marshall, A. C., Benson, C. B., Silva, V., Tworetzky, W. 2010; 27 (1): 40-45

  Abstract

  Fetal cardiac intervention (FCI) has been performed at our center in selected fetuses with complex congenital heart disease since 2000. Most interventions are performed in fetuses with a ductus arteriosus (DA)-dependent circulation. Indomethacin promotes closure of the DA in newborns and in fetal life, a potentially life threatening complication in fetuses with ductus-dependent congenital heart disease.We reviewed our experience with FCI with a focus on the frequency, features, and clinical course of ductal constriction. Fetuses undergoing FCI receive comprehensive pre- and postoperative cardiac and cerebral ultrasound evaluation, approximately 24 hours before and after the procedure, including imaging of DA flow and Doppler assessment of the umbilical artery and vein, ductus venosus, and, since 2004, the middle cerebral artery.Among 113 fetuses that underwent FCI, 24 of which were older than 28 0/7 weeks gestation, 2 were found to have DA constriction due to indomethacin therapy within 24 hours of intervention. Both of these were 30-week fetuses with hypoplastic left heart syndrome and restrictive or intact atrial septum. The DA was stenotic by spectral and color Doppler, and middle cerebral and umbilical artery pulsatility indexes were depressed. After discontinuation of indomethacin, the Doppler indices improved or normalized.Close echocardiographic monitoring of fetal Doppler flow velocities is very important after fetal intervention and indomethacin treatment, as the consequences of DA constriction in a fetus with hypoplastic left heart syndrome are potentially lethal. Sonographic evaluation should include measurement of cerebral and umbilical arterial flow velocities as well as color and spectral Doppler interrogation of the DA.

  View details for DOI 10.1159/000268290

  View details for Web of Science ID 000274051800007

  View details for PubMedID 20016136

• CEREBRAL BLOOD FLOW CHARACTERISTICS AND BIOMETRY IN FETUSES UNDERGOING PRENATAL INTERVENTION FOR AORTIC STENOSIS WITH EVOLVING HYPOPLASTIC LEFT HEART SYNDROME ULTRASOUND IN MEDICINE AND BIOLOGY McElhinney, D. B., Benson, C. B., Brown, D. W., Wilkins-Haug, L. E., Marshall, A. C., Zaccagnini, L., Tworetzky, W. 2010; 36 (1): 29-37

  Abstract

  Children with hypoplastic left heart syndrome (HLHS) are at risk for neurodevelopmental dysfunction; prenatal factors may play a role in this predilection. Cerebral blood flow profiles are abnormal in fetuses with HLHS, raising the possibility that cerebral hemodynamics in utero may be related to neurodevelopmental abnormalities. Prenatal aortic valvuloplasty for fetal aortic stenosis with evolving HLHS is technically feasible and improves left heart hemodynamics. This study aimed to assess the effects of prenatal intervention on cerebral blood flow profiles and head circumference in fetuses with evolving HLHS. Seventy fetuses underwent prenatal aortic valvuloplasty for evolving HLHS (median 23 weeks gestation). Among 46 fetuses that had successful valvuloplasty and available data, middle cerebral artery (MCA) pulsatility (PI) and resistive (RI) indices were abnormal (Z-scores -1.7+/-1.1 and -2.2+/-1.4, p<0.001). Early post-valvuloplasty (n=33) and at late gestation follow-up (n=28), MCA PI and RI Z-scores remained low with no difference from pre- or early postintervention. Fetal head circumference was normal, as were umbilical artery PI and RI Z-scores. Cerebral blood flow characteristics are abnormal in mid-gestation fetuses with evolving HLHS, suggesting low cerebral vascular impedance. The mechanisms and significance of these abnormalities are unknown. Prenatal aortic valvuloplasty did not have a major impact on these indices. (E-mail: doff.mcelhinney@cardio.chboston.org).

  View details for DOI 10.1016/j.ultrasmedbio.2009.09.004

  View details for Web of Science ID 000278012200005

  View details for PubMedID 19931971

• Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Emani, S. M., Bacha, E. A., McElhinney, D. B., Marx, G. R., Tworetzky, W., Pigula, F. A., del Nido, P. J. 2009; 138 (6): 1276-1282

  Abstract

  Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation--endocardial fibroelastosis resection with mitral or aortic valvuloplasty--on left ventricular function and clinical outcomes.All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test.Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1-38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively.In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group.

  View details for DOI 10.1016/j.jtcvs.2009.08.009

  View details for Web of Science ID 000272029800002

  View details for PubMedID 19819471

• Implantation of the Melody Transcatheter Pulmonary Valve in Patients With a Dysfunctional Right Ventricular Outflow Tract Conduit Early Results From the US Clinical Trial JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Zahn, E. M., Hellenbrand, W. E., Lock, J. E., McElhinney, D. B. 2009; 54 (18): 1722-1729

  Abstract

  This study was designed to evaluate the safety, procedural success, and short-term effectiveness of the Melody transcatheter pulmonary valve (Medtronic, Inc., Minneapolis, Minnesota) in patients with dysfunctional right ventricular outflow tract conduits.Conduit dysfunction has recently been treated with transcatheter pulmonary valve placement. There have been no prospective, multicenter trials evaluating this technology.Standardized entry criteria, implantation, and follow-up protocols were used. Nonimplanting core laboratories were used to evaluate results.Between January 2007 and September 2007, 34 patients underwent catheterization for intended Melody valve implantation at 3 centers. Mean age was 19.4 +/- 7.7 years. Initial conduit Doppler mean gradient was 28.8 +/- 10.1 mm Hg, and 94% of patients had moderate or severe pulmonary regurgitation (PR). Implantation was successful in 29 of 30 attempts and not attempted in 4 patients. Procedural complications included conduit rupture requiring urgent surgery and device removal (n = 1), wide-complex tachycardia (n = 1), and distal pulmonary artery guidewire perforation (n = 1). Peak systolic conduit gradient fell acutely from 37.2 +/- 16.3 mm Hg to 17.3 +/- 7.3 mm Hg, and no patient had more than mild PR. There were no deaths or further device explants. At 6-month follow-up, conduit Doppler mean gradient was 22.4 +/- 8.1 mm Hg, and PR fraction by magnetic resonance imaging was significantly improved (3.3 +/- 3.6% vs. 27.6 +/- 13.3%, p < 0.0001). Stent fracture occurred in 8 of 29 implants; 3 of these were treated with a second Melody valve for recurrent stenosis later in follow-up.Implantation of the Melody valve for right ventricular outflow tract conduit dysfunction can be performed by experienced operators at multiple centers, appears safe, and has encouraging acute and short-term outcomes.

  View details for DOI 10.1016/j.jacc.2009.06.034

  View details for Web of Science ID 000270971400011

  View details for PubMedID 19850214

• Predictors of Technical Success and Postnatal Biventricular Outcome After In Utero Aortic Valvuloplasty for Aortic Stenosis With Evolving Hypoplastic Left Heart Syndrome CIRCULATION McElhinney, D. B., Marshall, A. C., Wilkins-Haug, L. E., Brown, D. W., Benson, C. B., Silva, V., Marx, G. R., Mizrahi-Arnaud, A., Lock, J. E., Tworetzky, W. 2009; 120 (15): 1482-U48

  Abstract

  Aortic stenosis in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings. Prenatal balloon aortic valvuloplasty may improve left heart growth and function, possibly preventing evolution to hypoplastic left heart syndrome.Between March 2000 and October 2008, 70 fetuses underwent attempted aortic valvuloplasty for critical aortic stenosis with evolving hypoplastic left heart syndrome. We analyzed this experience to determine factors associated with procedural and postnatal outcome. The median gestational age at intervention was 23 weeks. The procedure was technically successful in 52 fetuses (74%). Relative to 21 untreated comparison fetuses, subsequent prenatal growth of the aortic and mitral valves, but not the left ventricle, was improved after intervention. Nine pregnancies (13%) did not reach a viable term or preterm birth. Seventeen patients had a biventricular circulation postnatally, 15 from birth. Larger left heart structures and higher left ventricular pressure at the time of intervention were associated with biventricular outcome. A multivariable threshold scoring system was able to discriminate fetuses with a biventricular outcome with 100% sensitivity and modest positive predictive value.Technically successful aortic valvuloplasty alters left heart valvar growth in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome and, in a subset of cases, appeared to contribute to a biventricular outcome after birth. Fetal aortic valvuloplasty carries a risk of fetal demise. Fetuses undergoing in utero aortic valvuloplasty with an unfavorable multivariable threshold score at the time of intervention are very unlikely to achieve a biventricular circulation postnatally.

  View details for DOI 10.1161/CIRCULATIONAHA.109.848994

  View details for Web of Science ID 000270706500005

  View details for PubMedID 19786635

• In Utero Valvuloplasty for Pulmonary Atresia With Hypoplastic Right Ventricle: Techniques and Outcomes PEDIATRICS Tworetzky, W., McElhinney, D. B., Marx, G. R., Benson, C. B., Brusseau, R., Morash, D., Wilkins-Haug, L. E., Lock, J. E., Marshall, A. C. 2009; 124 (3): E510-E518

  Abstract

  Prenatal intervention for fetuses with pulmonary atresia with an intact ventricular septum (PA/IVS) has the potential to alter right heart physiologic features in utero, facilitating right heart growth and improving the prospect of a biventricular outcome after birth.Since 2002, we have considered prenatal intervention for fetal PA/IVS in patients with (1) membranous pulmonary atresia, with identifiable pulmonary valve (PV) leaflets or membrane; (2) an intact or highly restrictive ventricular septum; and (3) right heart hypoplasia, with a tricuspid valve annulus z score of -2 or below and an identifiable but small right ventricle. Intervention was performed through direct cardiac puncture under ultrasound guidance, with percutaneous access or access through a limited laparotomy.Ten fetuses underwent attempted balloon dilation of the PV in utero. The first 4 procedures were technically unsuccessful, and the most-recent 6 were technically successful. Compared with control fetuses with PA/IVS who did not undergo prenatal intervention and had univentricular outcomes after birth, the tricuspid valve annulus, right ventricle length, and PV annulus grew significantly more from midgestation to late gestation in the 6 fetuses who underwent successful interventions.In utero perforation and dilation of the PV in midgestation fetuses with PA/IVS is technically feasible and may be associated with improved right heart growth and postnatal outcomes for fetuses with moderate right heart hypoplasia in midgestation. There is an important learning curve for this procedure, and much remains to be learned about the selection of appropriate fetuses for prenatal intervention.

  View details for DOI 10.1542/peds.2008-2014

  View details for Web of Science ID 000269383100042

  View details for PubMedID 19706566

• Exercise Function of Children with Congenital Aortic Stenosis Following Aortic Valvuloplasty during Early Infancy CONGENITAL HEART DISEASE Kipps, A. K., McElhinney, D. B., Kane, J., Rhodes, J. 2009; 4 (4): 258-264

  Abstract

  The objectives of this study were to characterize the exercise function of patients treated with balloon aortic valvuloplasty at 6 years old with a history of neonatal AS to undergo exercise testing.We enrolled 30 patients (median age 13.1 years) who underwent balloon aortic valvuloplasty at a median age of 12 days. At time of exercise testing, the median maximum Doppler AS gradient was 34 mm Hg (0-70 mm Hg); 11 patients had moderate or severe aortic regurgitation. All patients were asymptomatic. Overall, peak oxygen consumption (VO(2)) was below normal (87 +/- 18% predicted; P < .001), and was severely depressed (

  View details for DOI 10.1111/j.1747-0803.2009.00304.x

  View details for Web of Science ID 000207893700007

  View details for PubMedID 19664028

  View details for PubMedCentralID PMC4269337

• Effectiveness of Balloon Valvuloplasty for Palliation of Mitral Stenosis After Repair of Atrioventricular Canal Defects AMERICAN JOURNAL OF CARDIOLOGY Robinson, J. D., Marx, G. R., del Nido, P. J., Lock, J. E., McElhinney, D. B. 2009; 103 (12): 1770-1773

  Abstract

  Closure of a mitral valve (MV) cleft, small left-sided cardiac structures, and ventricular imbalance all may contribute to mitral stenosis (MS) after repair of atrioventricular canal (AVC) defects. MV replacement is the traditional therapy but carries high risk in young children. The utility of balloon mitral valvuloplasty (BMV) in postoperative MS is not established and may offer alternative therapy or palliation. Since 1996, 10 patients with repaired AVC defects have undergone BMV at a median age of 2.5 years (range 8 months to 14 years), a median of 2 years after AVC repair. At catheterization, the median value of mean MS gradients was 16 mm Hg (range 12 to 22) and was reduced by 34% after BMV. Before BMV, there was mild mitral regurgitation in 9 of 10 patients, which increased to severe in 1 patient. All patients were alive at follow-up (median 5.4 years). Repeat BMV was performed in 4 patients, 10 weeks to 18 months after initial BMV. One patient underwent surgical valvuloplasty; 3 underwent MV replacement 2, 3, and 28 months after BMV. In the 6 patients (60%) with a native MV at most recent follow-up (median 3.2 years), the mean Doppler MS gradient was 9 mm Hg, the median weight had doubled, and weight percentile had increased significantly. In conclusion, BMV provides relief of MS in most patients with repaired AVC defects; marked increases in mitral regurgitation are uncommon. Because BMV can incompletely relieve obstruction and increase mitral regurgitation, it will not be definitive in most patients but will usually delay MV replacement to accommodate a larger prosthesis.

  View details for DOI 10.1016/j.amjcard.2009.02.062

  View details for Web of Science ID 000267407000025

  View details for PubMedID 19539091

• Truncus Arteriosus: Diagnostic Accuracy, Outcomes, and Impact of Prenatal Diagnosis PEDIATRIC CARDIOLOGY Swanson, T. M., Tierney, E. S., Tworetzky, W., Pigula, F., McElhinney, D. B. 2009; 30 (3): 256-261

  Abstract

  Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at

  View details for DOI 10.1007/s00246-008-9328-7

  View details for Web of Science ID 000264884500007

  View details for PubMedID 19015910

• Ultra-High-Pressure Balloon Angioplasty for Treatment of Resistant Stenoses Within or Adjacent to Previously Implanted Pulmonary Arterial Stents CIRCULATION-CARDIOVASCULAR INTERVENTIONS Maglione, J., Bergersen, L., Lock, J. E., McElhinney, D. B. 2009; 2 (1): 52-58

  Abstract

  Stents are essential tools in the management of pulmonary arterial (PA) stenosis in patients with congenital heart disease. Although stents can usually be reexpanded as children grow, resistant in-stent or peri-stent obstruction can complicate the management of PA stents. Angioplasty with ultra-high-pressure (UHP) balloons may facilitate successful treatment of stent-associated PA stenoses that are resistant to high-pressure dilation.We reviewed patients who underwent UHP angioplasty of in-stent or peri-stent PA stenoses that were resistant to high-pressure redilation. A resistant stenosis was defined as a residual balloon waist during high-pressure redilation of the stent, along with a pressure gradient and/or angiographic stenosis. Thirty-four lesions in 29 patients, including 8 with multiple concentric, overlapping, or adjacent stents, were included. The median age at UHP angioplasty was 9 years, and a median of 4 years had elapsed since unsuccessful high-pressure angioplasty. Thirty-one of the 34 (91% [81% to 100%]) UHP angioplasty procedures were successful in relieving the resistant stenosis. Balloon:waist diameter ratios were conservative (median 1.26), reflecting the ability of UHP balloons to "fracture" nearly all obstructions. After UHP dilation, lesion diameter increased by a median of 3.1 mm (36%), significantly more than after previous high-pressure dilation (1.3 mm, 19%; P<0.001). In 5 lesions, UHP angioplasty fractured the stent, allowing further vessel expansion. There were no vascular or other complications.UHP angioplasty was safe and effective for treatment of stent-related resistant PA stenosis in this series; the ability to fracture maximally expanded stents may extend the utility of stents in the pediatric population.

  View details for DOI 10.1161/CIRCINTERVENTIONS.108.826263

  View details for Web of Science ID 000276048500009

  View details for PubMedID 20031693

• Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome PRENATAL DIAGNOSIS Marshall, A. C., Levine, J., Morash, D., Silva, V., Lock, J. E., Benson, C. B., Wilkins-Haug, L. E., McElhinney, D. B., Tworetzky, W. 2008; 28 (11): 1023-1028

  Abstract

  Neonates with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have a high rate of mortality. We sought to assess the effect of prenatal intervention intended to create atrial septal defects in fetuses with this diagnosis.We reviewed the medical records and imaging of all fetuses undergoing intervention for atrial septal defect creation in the setting of hypoplastic left heart syndrome and intact atrial septum. The procedures were performed with a percutaneous cardiac puncture under the guidance of ultrasonography. For the 21 interventions, patient and procedural characteristics were analyzed to identify predictors of neonatal outcome.Of 21 procedures attempted between 24 and 34 weeks' gestation, 19 were technically successful. Fetal demise occurred in two cases. The size of the created defect varied and measured at least 3 mm in six fetuses. Among 19 neonates, a larger atrial septal defect was associated with higher oxygen saturation and less need for intervention prior to surgical single-ventricle palliation.Technically successful atrial septal defect creation in fetuses with hypoplastic left heart syndrome and intact atrial septum results in atrial septal defects of varying size; defects of at least 3 mm in diameter appear to confer postnatal benefit.

  View details for DOI 10.1002/pd.2114

  View details for Web of Science ID 000261125700007

  View details for PubMedID 18925607

• Mitral valve replacement in infants and children 5 years of age or younger: Evolution in practice and outcome over three decades with a focus on supra-annular prosthesis implantation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Tierney, E. S., Pigula, F. A., Berul, C. I., Lock, J. E., del Nido, P. J., McElhinney, D. B. 2008; 136 (4): 954-U8

  Abstract

  Successful mitral valve replacement in young children is limited by the lack of small prosthetic valves. Supra-annular prosthesis implantation can facilitate mitral valve replacement with a larger prosthesis in children with a small annulus, but little is known about its effect on the outcomes of mitral valve replacement in young children.One hundred eighteen children underwent mitral valve replacement at 5 years of age or younger from 1976-2006. Mitral valve replacement was supra-annular in 37 (32%) patients.Survival was 74% +/- 4% at 1 year and 56% +/- 5% at 10 years but improved over time (10-year survival of 83% +/- 7% from 1994-2006). Factors associated with worse survival included earlier mitral valve replacement date, age less than 1 year, complete atrioventricular canal, and additional procedures at mitral valve replacement, but not supra-annular mitral valve replacement. As survival improved during our more recent experience, the risks of supra-annular mitral valve replacement became apparent; survival was worse among patients with a supra-annular prosthesis after 1991. A pacemaker was placed in 18 (15%) patients within 1 month of mitral valve replacement and was less likely in patients who had undergone supra-annular mitral valve replacement. Among early survivors, freedom from redo mitral valve replacement was 72% +/- 5% at 5 years and 45% +/- 7% at 10 years. Twenty-one patients with a supra-annular prosthesis underwent redo mitral valve replacement. The second prosthesis was annular in 15 of these patients and upsized in all but 1, but 5 required pacemaker placement for heart block.Supra-annular mitral valve replacement was associated with worse survival than annular mitral valve replacement in our recent experience. Patients with supra-annular mitral valve replacement were less likely to have operative complete heart block but remained at risk when the prosthesis was subsequently replaced.

  View details for DOI 10.1016/j.jtcvs.2007.12.076

  View details for Web of Science ID 000260314800023

  View details for PubMedID 18954636

• In situ fracture of stents implanted for relief of pulmonary arterial stenosis in patients with congenitally malformed hearts CARDIOLOGY IN THE YOUNG McElhinney, D. B., Bergersen, L., Marshall, A. C. 2008; 18 (4): 405-414

  Abstract

  One of the most common uses of stents in patients with congenitally malformed hearts is treatment of pulmonary arterial stenosis. Although there are reports of fractured pulmonary arterial stents, little is known about the risk factors for, and implications of, such fractures.We reviewed angiograms to identify fractures in stents previously inserted to relieve stenoses in pulmonary arteries from 1990 through 2001 in patients who also underwent follow-up catheterization at least 3 years after placement of the stent. We undertook matched cohort analysis, matching a ratio of 2 fractured to 1 unfractured stent.Overall, 166 stents meeting the criterions of our study had been placed in 120 patients. We identified fractures in 35 stents (21%) in 29 patients. All fractured stents were in the central pulmonary arteries, 24 (69%) in the central part of the right pulmonary artery, and all were complete axial fractures, or complex fractures along at least 2 planes. Stent-related factors associated with increased risk of fracture identified by multivariable logistic regression included placement in close apposition to the ascending aorta (p = 0.001), and a larger expanded diameter (p = 0.002). There was obstruction across 28 of 35 fractured stents, which was severe in 11. We re-stented 21 of the fractured stents, and recurrent fracture was later diagnosed in 3 of these. A fragment of the fractured stent embolized distally in 2 patients, without clinically important effects.In situ fracture of pulmonary arterial stents is relatively common, and in most cases is related to compression by the aorta. There is usually recurrent obstruction across the fractured stent, but fractured stents rarely embolize, and are not associated with other significant complications.

  View details for DOI 10.1017/S1047951108002424

  View details for Web of Science ID 000258393300009

  View details for PubMedID 18559137

• Echocardiographic predictors of mitral stenosis-related death or intervention in infants AMERICAN HEART JOURNAL Tierney, E. S., Graham, D. A., McElhinney, D. B., Trevey, S., Freed, M. D., Colan, S. D., Geva, T. 2008; 156 (2): 384-390

  Abstract

  The purpose of this study is to identify independent echocardiographic predictors of mitral stenosis (MS)-related death or intervention in infants. Congenital MS is a rare and morphologically heterogeneous lesion with variable prognosis. Among patients diagnosed with MS in early infancy, echocardiographic factors associated with MS-related intervention or death have not been determined.The clinical and echocardiographic data of patients diagnosed with MS at age <6 months by echocardiography between 1986 and 2004 were reviewed. The primary outcome was a composite end point of either mitral valve (MV) intervention (catheter or surgery) or death related to MS. Multiple variables from the initial echocardiogram were analyzed for association with outcomes. Seventy-one patients (median age at diagnosis 63 days) fulfilled the inclusion criteria. Multivariate analysis identified higher initial MV mean inflow gradient (P = .009) and lower left ventricular (LV) diastolic length Z-score (P = .006) at presentation as predictors of intervention or death. Among patients with an initial MV inflow gradient < 2 mm Hg, none reached an end point, whereas, among patients with an initial mean gradient >/= 5.5 mm Hg, the risk of intervention or death was 85%. Among patients with a gradient > 2 and < 5.5 mm Hg, an end point was reached in 38%, and an LV diastolic length Z-score

  View details for DOI 10.1016/j.ahj.2008.03.019

  View details for Web of Science ID 000258333400034

  View details for PubMedID 18657675

• Outcome of fetuses and infants with double inlet single left ventricle AMERICAN JOURNAL OF CARDIOLOGY Tham, E. B., Wald, R., McElhinney, D. B., Hirji, A., Goff, D., del Nido, P. J., Hornberger, L. K., Nield, L. E., Tworetzky, W. 2008; 101 (11): 1652-1656

  Abstract

  Double-inlet left ventricle (DILV) includes a diverse range of anatomic variables that affect the surgical strategy. The aim of this study was to determine the impact of anatomic subtype, associated anomalies, and fetal diagnosis on the management and outcomes of fetuses and infants with DILV. The outcomes of fetuses and infants with DILV diagnosed from 1990 to 2004 at 3 major referral centers were reviewed. Sixty-five cases of DILV were detected prenatally. Twenty-one of these pregnancies were terminated, including 17 of 37 (46%) in which the diagnoses were made at < or =24 weeks of gestation. An additional 106 patients were diagnosed with DILV within the first 3 months of life. The percentage of patients diagnosed prenatally increased significantly over the study period. Transplantation-free survival was 88%, 82%, 79%, and 76% at 1 month, 1 year, 5 years, and 10 years, respectively. Factors associated with improved survival in univariate analysis included year of birth after 1994, no neonatal Norwood or Damus procedure, and no neonatal surgery at all. In multivariate analysis, any neonatal surgery was the only factor associated with worse survival. Associated anomalies and prenatal diagnosis were not associated with postnatal outcome. In conclusion, although the frequency of prenatal diagnosis of DILV has increased significantly over the past 15 years, prenatal diagnosis is not associated with better postnatal survival. During this same period, postnatal survival has improved substantially. Neonatal surgery of any type was the only independent risk factor for worse survival.

  View details for DOI 10.1016/j.amjcard.2008.01.048

  View details for Web of Science ID 000256450700020

  View details for PubMedID 18489946

• Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome PEDIATRIC CARDIOLOGY Foerster, S. R., Gauvreau, K., McElhinney, D. B., Geva, T. 2008; 29 (3): 536-544

  Abstract

  Historically, outcomes of patients with heterotaxy syndrome and congenital heart disease have been poor. Published series include patients treated over many decades or focus on specific patient/operative subgroups. This study was performed to evaluate midterm outcomes and determine anatomic risk factors for death in patients with all types of heterotaxy syndrome treated after 1985. We reviewed all infants with heterotaxy born between 1985 and 1997, presenting to one institution at <6 months age, followed for > or =5 years. Of 102 study patients (46 with asplenia phenotype, 56 with polysplenia phenotype), 48 (47%) died at a median age of 0.6 months, 12 without intervention. Survivors were followed for a median of 12.8 years (5-21.7 years). Independent predictors of mortality included obstructed totally anomalous pulmonary venous connection (TAPVC) (OR, 7.8; 95% CI, 1.9-32.9; p = 0.005), mild or greater atrioventricular (AV) valve regurgitation at presentation (OR, 3.5; 95% CI, 1.0-12.1; p = 0.03), and common AV canal (OR, 3.1; 95% CI, 1.1-8.5; p = 0.03). Sixteen patients developed pulmonary vein stenosis at a median age of 2 months, with 5 (31%) alive at follow-up. In patients with TAPVC, the mean indexed sum of pulmonary vein diameters was lower among patients who died than in survivors (42.3 +/- 8.3 vs 49.5 +/- 10.1 mm/m2; p = 0.02). Mortality remains high among patients with heterotaxy treated in the modern surgical era, particularly those with obstructed TAPVC. Pulmonary vein stenosis is common after repair of TAPVC in patients with heterotaxy, may be diagnosed beyond infancy, and is associated with poor outcomes.

  View details for DOI 10.1007/s00246-007-9128-5

  View details for Web of Science ID 000255754100010

  View details for PubMedID 18004616

• Surgical aortic valvuloplasty in children and adolescents with aortic regurgitation: Acute and intermediate effects on aortic valve function and left ventricular dimensions JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bacha, E. A., McElhinney, D. B., Guleserian, K. J., Colan, S. D., Jonas, R. A., del Nido, P. J., Marx, G. R. 2008; 135 (3): 552-U38

  Abstract

  Surgical aortic valvuloplasty is increasingly employed in the management of children and adolescents with aortic regurgitation, but the durability of this approach and factors associated with outcome are not well defined.From 1989 to 2005, a total of 81 patients younger than 19 years with moderate or severe aortic regurgitation underwent surgical aortic valvuloplasty. Aortic regurgitation was congenital in 20 cases, after treatment of aortic stenosis in 30, from other injuries to the aortic valve in 12, and from other causes in 19. Eighteen patients had moderate or severe aortic stenosis. Preoperative left ventricular end-diastolic dimension z score was 4.9 +/- 2.7.After surgical aortic valvuloplasty with various techniques, including pericardial leaflet augmentation in 80%, aortic regurgitation was improved in 77 patients and was mild or less in 68. Ten of 18 patients with moderate or severe aortic stenosis before repair had a decrease to mild, whereas 2 had progression from mild to moderate. Left ventricular end-diastolic dimension z score decreased by 2.9 +/- 2.1 (P < .001). During follow-up (median 4.7 years), 33 patients underwent aortic valve reinterventions, including aortic valve replacement in 25. Estimated freedoms from aortic valve replacement were 72% +/- 6% at 5 years and 54% +/- 9% at 7.5 years and were shorter in patients with moderate or severe aortic stenosis before surgical aortic valvuloplasty. Among surviving patients who did not undergo aortic valve replacement, aortic regurgitation at follow-up was moderate in 21 and trivial or mild in 34; left ventricular and aortic root dimensions were preserved.Surgical aortic valvuloplasty is a valid option with good intermediate results for children and adolescents with aortic regurgitation from a variety of causes, particularly for patients with less than moderate aortic stenosis.

  View details for DOI 10.1016/j.jtcvs.2007.09.057

  View details for Web of Science ID 000253860300015

  View details for PubMedID 18329469

• Changes in left heart hemodynamics after technically successful in-utero aortic valvuloplasty ULTRASOUND IN OBSTETRICS & GYNECOLOGY Tierney, E. S., Wald, R. M., McElhinney, D. B., Marshall, A. C., Benson, C. B., Colan, S. D., Marcus, E. N., Marx, G. R., Levine, J. C., Wilkins-Haug, L., Lock, J. E., Tworetzky, W. 2007; 30 (5): 715-720

  Abstract

  Severe aortic stenosis in the mid-gestation fetus can progress to hypoplastic left heart syndrome (HLHS). @ In-utero aortic valvuloplasty is an innovative therapy to promote left ventricular growth and function and potentially to prevent HLHS. This study evaluated the effects of mid-gestation fetal balloon aortic valvuloplasty on subsequent fetal left ventricular function and left heart Doppler characteristics.We reviewed fetuses with aortic stenosis that underwent attempted in-utero aortic valvuloplasty between 2000 and 2006. Pre-intervention and the latest post-intervention fetal echocardiograms were analyzed to characterize changes in left heart function and Doppler characteristics in utero.Forty-two fetuses underwent attempted aortic valvuloplasty during the study period, 12 of which were excluded from analysis secondary to inadequate follow-up data, termination or fetal demise. Study fetuses (n = 30) underwent pre-intervention echocardiography at a median gestational age of 23 weeks, and were followed for a median of 66 +/- 23 days post-intervention. In 26 fetuses, aortic valvuloplasty was technically successful. Among these 26, left heart physiology was abnormal pre-intervention and improved or normalized after intervention in most cases: biphasic mitral inflow was present in 5/25 (20%) cases pre-intervention and in 21/23 (91%) post-intervention (P < 0.001); moderate or severe mitral regurgitation was present in 14/26 (54%) cases pre-intervention and in 5/23 (22%) post-intervention (P = 0.02); bidirectional flow across the patent foramen ovale was present in 0/26 cases pre-intervention and in 6/25 (24%) post-intervention (P = 0.01); antegrade flow in the transverse arch was present in 0/25 cases pre-intervention and in 17/26 (65%) post-intervention (P < 0.001). The left ventricular ejection fraction increased from 19 +/- 10% pre-intervention to 39 +/- 14% post-intervention (P < 0.001). These changes were not observed in control fetuses (n = 18).Fetal aortic valvuloplasty, when technically successful, improves left ventricular systolic function and left heart Doppler characteristics.

  View details for DOI 10.1002/uog.5132

  View details for Web of Science ID 000250732900006

  View details for PubMedID 17764106

• Pathophysiology, management, and outcomes of fetal hemodynamic instability during prenatal cardiac intervention PEDIATRIC RESEARCH Mizrahi-Arnaud, A., Tworetzky, W., Bulich, L. A., Wilkins-Haug, L. E., Marshall, A. C., Benson, C. B., Lock, J. E., McElhinney, D. B. 2007; 62 (3): 325-330

  Abstract

  Prenatal cardiac intervention (PCI) may favorably alter the in utero course of some congenital heart defects. In our preliminary experience with PCI, fetal hemodynamic instability (FHI) characterized by bradycardia and ventricular dysfunction was common. This study evaluated the pathophysiology, management, and short-term outcomes of FHI during PCI for aortic stenosis with evolving hypoplastic left heart syndrome (HLHS), HLHS with restrictive atrial septum, pulmonary atresia with intact ventricular septum, and hydrops due to structural heart disease. From 2000 to 2006, 83 fetuses underwent PCI, with ventricular access in 63, atrial access in 17, and both in three. FHI occurred in 37 fetuses (45%). FHI was associated with transventricular PCI (all but one case of FHI; p < 0.001) and large hemopericardium (n=9; p=0.07). Prolonged FHI was associated with severe ventricular distortion during ventricular puncture (p = 0.06). FHI was treated with resuscitation medications in 31 of 37 fetuses and resolved in all 37. Five fetuses died within 1 d of PCI: four had FHI and one had a massive hemopericardium. FHI is common and clinically important during transventricular PCI and may be caused by a ventricular reflex or reduced cardiac output from cardiac distortion during ventricular puncture. Hemopericardium may be causative in a subset of fetuses.

  View details for Web of Science ID 000248973200018

  View details for PubMedID 17622948

• Outcome after Fontan failure and takedown to an intermediate palliative circulation ANNALS OF THORACIC SURGERY Almond, C. S., Mayer, J. E., Thiagarajan, R. R., Blume, E. D., del Nido, P. J., McElhinney, D. B. 2007; 84 (3): 880-887

  Abstract

  Fontan takedown to an intermediate palliative circulation is an important treatment option for patients with acute or subacute failure of a Fontan circulation from a variety of causes. Little is known about the subsequent outcome of these patients or their potential candidacy for a second attempt at Fontan completion.Patients followed up at Children's Hospital Boston who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 1 year of Fontan completion were reviewed.Between 1979 and 2006, 53 patients underwent Fontan takedown at a median age of 2.3 years (range, 0.3 to 36.5 years). Takedown was performed during the Fontan procedure itself in 12 patients (22%), within the first postoperative month in 31(58%), and between 1 month and 1 year in 10 (18%). Overall, 29 patients (55%) survived the early period after takedown, and 19 ultimately underwent successful Fontan completion a median of 4.6 years after takedown; all but one was alive a median of 6.4 years later. Thirteen (68%) of the 19 had treatable abnormalities contributing to Fontan failure.Fontan takedown can provide effective stabilization of the acutely or subacutely failing Fontan circulation, although a substantial number of patients die early despite Fontan takedown. Subjects surviving the perioperative period can often undergo uneventful redo Fontan. A thorough evaluation for treatable abnormalities should be performed in all patients with a failing Fontan circulation and in patients who undergo Fontan takedown.

  View details for DOI 10.1016/j.athoracsur.2007.02.092

  View details for Web of Science ID 000248982400025

  View details for PubMedID 17720394

• Acute and intermediate outcomes, and evaluation of injury to the aortic wall, as based on 15 years experience of implanting stents to treat aortic coarctation CARDIOLOGY IN THE YOUNG Qureshi, A. M., McElhinney, D. B., Lock, J. E., Landzberg, M. J., Lang, P., Marshall, A. C. 2007; 17 (3): 307-318

  Abstract

  Stenting for aortic coarctation has been shown to be effective in the short term. The safety and longer term efficacy of transcatheter therapy, however, must be well established if the technique is to be widely accepted as an alternative to surgery. In order to determine the frequency, spectrum, and outcome of injury to the aortic wall caused by angioplasty or stenting of aortic coarctation, the nomenclature of mural injury in these patients must be adapted to the conditions of transcatheter therapy.Between 1989 and July 2005, we inserted stents in 153 patients with aortic coarctation, their median age being 15.8 years. Prior aortic interventions had been performed in 98 patients, and preexisting aneurysms were observed in 19. Stenting resulted in a significant reduction of the gradient across the site of coarctation, from a median of 30 millimetres of mercury to zero (p less than 0.001), with a residual gradient within the aortic arch of 20 millimetres of mercury or more in 5% of patients. Acute injuries to the aortic wall, other than therapeutic tears, were observed in 3 patients (2%), none of whom required surgery. At median follow-up of 2.5 years, this being more than 5 years in 30 patients, 4 patients had died, albeit none from complications relating to stenting or catheterization. Acute injuries to the aortic wall did not progress, and new aneurysms were observed in 6% of patients subsequent to follow-up imaging. Stent fractures, and jailed or partially covered brachiocephalic vessels, were observed in 12, and 49, patients, respectively, but did not result in haemodynamic or embolic complications.Stenting for aortic coarctation results in consistent relief of the gradient, and few serious complications in the short and intermediate term. Serious injuries to the aortic wall are uncommon in our experience, and can be minimized with a focus on technical measures, such as pre-dilation before stenting.

  View details for DOI 10.1017/S1047951107000339

  View details for Web of Science ID 000247220000011

  View details for PubMedID 17319978

• Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum PEDIATRICS Salvin, J. W., McElhinney, D. B., Colan, S. D., Gauvreau, K., del Nido, P. J., Jenkins, K. J., Lock, J. E., Tworetzky, W. 2006; 118 (2): E415-E420

  Abstract

  Pulmonary atresia with intact ventricular septum is a complex congenital cardiovascular anomaly that frequently requires single ventricle palliation. Fetal diagnosis of pulmonary atresia with intact ventricular septum is common, but the natural history of pulmonary atresia with intact ventricular septum diagnosed in midgestation, predictors of neonatal anatomy, and predictors of biventricular repair have not been determined. The objective of this study was to determine whether the size and rate of growth of the fetal tricuspid valve predict neonatal anatomy and biventricular repair.Twenty-three fetuses diagnosed with pulmonary atresia with intact ventricular septum between 1990 and 2004 were studied. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 1 achieved biventricular repair, compared with 5 of 5 with a tricuspid valve z score > -3. Of 13 fetuses with a midgestation fetal tricuspid valve z score < or = -3, 8 were diagnosed postnatally with a right ventricular dependent coronary circulation, compared with none with a tricuspid valve z score > -3. Midgestation and late gestation fetal tricuspid valve z scores correlated with neonatal tricuspid valve z score. The average rate of tricuspid valve growth between mid- and late fetal echocardiograms was significantly lower in patients who did not achieve biventricular repair than in those who did (0.012 +/- 0.008 cm per week vs 0.028 +/- 0.014 cm per week).Fetal tricuspid valve z score and rate of growth predict postnatal outcome in pulmonary atresia with intact ventricular septum. These findings may have important implications for prenatal counseling and selection of patients for fetal pulmonary valve dilation.

  View details for DOI 10.1542/peds.2006-0428

  View details for Web of Science ID 000239440600099

  View details for PubMedID 16882782

• Validation and re-evaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Colan, S. D., McElhinney, D. B., Crawford, E. C., Keane, J. F., Lock, J. E. 2006; 47 (9): 1858-1865

  Abstract

  The purpose of this study was to validate and re-evaluate our previously reported scoring systems for predicting optimal management in neonates with aortic stenosis (AS).In 1991, we reported a multivariate discriminant equation and an ordinal scoring system for predicting which neonates with AS are suitable for biventricular repair and which are better served by single ventricle management.Retrospective analysis was performed to: 1) validate our scoring systems in 89 additional neonates with AS and normal mitral valve area, 2) assess the effects of 5% measurement variation on predictive scores, 3) evaluate our cohort with the Congenital Heart Surgeons' Society scoring system, and 4) repeat the discriminant analysis on the basis of all 126 patients.The original scores each predicted outcome accurately in 68 patients (77%). Minor (5%) measurement variation changed the outcome predicted by the discriminant equation in 8 patients (9%) and by the threshold system in 13 patients (15%). The most accurate model for predicting survival with a biventricular circulation among the full cohort is: 10.98 (body surface area) + 0.56 (aortic annulus z-score) + 5.89 (left ventricular to heart long-axis ratio) - 0.79 (grade 2 or 3 endocardial fibroelastosis) - 6.78. With a cutoff of -0.65, outcome was predicted accurately in 90% of patients.Both of our original scoring systems are less accurate at predicting outcome than in our original analysis. Revised discriminant analysis yielded a model similar to our original equation that was 90% accurate at predicting survival with a biventricular circulation among neonates with AS and a mitral valve area z-score >-2.

  View details for DOI 10.1016/j.jacc.2006.02.020

  View details for Web of Science ID 000237235500016

  View details for PubMedID 16682313

• Fetal aortic valve stenosis and the evolution of hypoplastic left heart syndrome - Patient selection for fetal intervention CIRCULATION Makikallio, K., McElhinney, D. B., Levine, J. C., Marx, G. R., Colan, S. D., Marshall, A. C., Lock, J. E., Marcus, E. N., Tworetzky, W. 2006; 113 (11): 1401-1405

  Abstract

  Fetal aortic valvuloplasty may prevent progression of aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). Predicting which fetuses with AS will develop HLHS is essential to optimize patient selection for fetal intervention. The aim of this study was to define echocardiographic features associated with progression of midgestation fetal AS to HLHS.Fetal echocardiograms were reviewed from 43 fetuses diagnosed with AS and normal left ventricular (LV) length at < or =30 weeks' gestation. Of 23 live-born patients with available follow-up data, 17 had HLHS and 6 had a biventricular circulation. At the time of diagnosis, LV length, mitral valve, aortic valve, and ascending aortic diameter Z-scores did not differ between fetuses that ultimately developed HLHS and those that maintained a biventricular circulation postnatally. However, all of the fetuses that progressed to HLHS had retrograde flow in the transverse aortic arch (TAA), 88% had left-to-right flow across the foramen ovale, 91% had monophasic mitral inflow, and 94% had significant LV dysfunction. In contrast, all 6 fetuses with a biventricular circulation postnatally had antegrade flow in the TAA, biphasic mitral inflow, and normal LV function. With advancing gestation, growth arrest of left heart structures became evident in fetuses developing HLHS.In midgestation fetuses with AS and normal LV length, reversed flow in the TAA and foramen ovale, monophasic mitral inflow, and LV dysfunction are predictive of progression to HLHS. These physiological features may help refine patient selection for fetal intervention to prevent the progression of AS to HLHS.

  View details for DOI 10.1161/CIRCULATIONAHA.105.588194

  View details for Web of Science ID 000236131700008

  View details for PubMedID 16534003

• Incorporation of the hepatic veins into the cavopulmonary circulation in patients with heterotaxy and pulmonary arteriovenous malformations after a Kawashima procedure ANNALS OF THORACIC SURGERY McElhinney, D. B., Kreutzer, J., Lang, P., Mayer, J. E., Del Nido, P. J., Lock, J. E. 2005; 80 (5): 1597-1603

  Abstract

  In patients with polysplenia syndrome and azygous continuation of an interrupted inferior vena cava (IVC), pulmonary arteriovenous malformations (PAVMs) are relatively common after bidirectional cavopulmonary anastomosis (BCPA, Kawashima procedure). Resolution of PAVMs after hepatic vein (HV) inclusion into the cavopulmonary circulation has been reported, but there has been no systematic investigation of the effects of this therapy in a population of more than 3 patients.We studied 16 patients with heterotaxy, univentricular congenital heart disease, and azygous continuation of the IVC who underwent incorporation of the HV into the cavopulmonary circuit for treatment of significant PAVMs after a Kawashima procedure.The median preoperative systemic arterial oxygen saturation (SsaO2) was 76% (65%-85%), compared with 89% (85% to 92%) early after BCPA. Among 15 early survivors, the median early postoperative SsaO2 was 76% (56%-85%). In 11 of the 15 survivors, SsaO2 rose to 90% or greater within a year and remained at 93% or greater at follow-up of 2.8 to 10 years. Four patients had persistent hypoxemia and residual PAVMs at follow-up catheterization 1.5 to 8 years postoperatively; these patients had the most severe hypoxemia prior to HV inclusion, and in 2 the residual PAVMs were unilateral, with HV flow streaming to the contralateral lung, in which PAVMs had resolved.Hypoxemia resolved after cavopulmonary incorporation of the HV in the majority of our patients with PAVMs after the Kawashima operation, presumably due to a combination of PAVM resolution and elimination of hepatic venoatrial right-to-left shunting. These findings support the theory that development of PAVMs is facilitated by exclusion of HV effluent from the pulmonary circulation.

  View details for DOI 10.1016/j.athoracsur.2005.05.101

  View details for Web of Science ID 000232970500005

  View details for PubMedID 16242423

• Creation of a brachial arteriovenous fistula for treatment of pulmonary arteriovenous malformations after cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Marshall, A. C., Lang, P., Lock, J. E., Mayer, J. E. 2005; 80 (5): 1604-1609

  Abstract

  Pulmonary arteriovenous malformations (PAVMs) occur in approximately 20% of patients after unidirectional superior cavopulmonary anastomosis (CPA), and frequently after bidirectional CPA in patients with polysplenia syndrome. It is hypothesized that exclusion of a growth-modulating factor produced in the liver may predispose to PAVM formation. Resolution of PAVMs after inclusion of hepatic venous effluent into the cavopulmonary circulation has been reported. An upper extremity systemic arteriovenous (AV) fistula may be created to augment pulmonary blood flow and improve oxygenation in hypoxemic patients with CPA, but there has been no systematic investigation of the effects of such fistulas on PAVMs after CPA.We studied 11 patients with PAVMs who underwent creation of a brachial AV fistula a median of 11 years after CPA.Eight patients had discontinuous pulmonary arteries or unilateral flow of a bidirectional CPA and were not considered good candidates for Fontan completion; the other 3 patients had polysplenia and unilateral hepatic venous streaming after Fontan completion. Three patients died of progressive complications of their heart disease 4 to 18 months after AV fistula creation. Pulmonary arteriovenous malformations resolved after creation of a brachial AV fistula in 4 of 5 surviving patients with unilateral flow of a superior CPA, but in none of 3 patients with polysplenia who had unilateral hepatic venous streaming after Fontan completion and PAVMs in the contralateral lung.These findings are consistent with the "hepatic factor" hypothesis, according to which the development of PAVMs is facilitated when an unidentified factor produced or metabolized in the liver does not reach the pulmonary circulation before traversing another capillary bed. Patients with unilateral superior CPA flow and PAVMs who are not considered candidates for Fontan completion may benefit from a brachial AV fistula.

  View details for DOI 10.1016/j.athoracsur.2005.05.100

  View details for Web of Science ID 000232970500006

  View details for PubMedID 16242424

• Improving outcomes in fetuses and neonates with congenital displacement (Ebstein's malformation) or dysplasia of the tricuspid valve AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Salvin, J. W., Colan, S. D., Thiagarajan, R., Crawford, E. C., Marcus, E. N., Del Nido, P. J., Tworetzky, W. 2005; 96 (4): 582-586

  Abstract

  From 1984 to 2004, 66 patients were diagnosed with Ebstein's malformation (n = 61) or congenital tricuspid valve (TV) dysplasia (n = 5) in utero or during the first month of life. Of these, 33 were diagnosed by fetal echocardiography at a median gestational age of 22 weeks, and 33 were diagnosed postnatally at a median age of 1 day (range 1 to 27). In 8 of the 33 prenatally diagnosed patients (24%), the pregnancies were terminated; in 9 (27%), the fetuses died in utero, and in 16 (49%), the fetuses survived to birth. Seven prenatally diagnosed patients survived beyond the neonatal period (21% of 33). Of the 49 neonates, 35 (71%) survived to hospital discharge and beyond 1 month of age. Independent predictors of death by multivariable logistic regression analysis included right atrial area index >1, the absence of anterograde flow across the pulmonary valve, and diagnosis before 1997. Although outcomes in fetuses and neonates with congenital anomalies of the TV have improved in more recent experience, survival in patients at the severe end of the spectrum remains poor. To improve outcomes in this group of high-risk patients, novel approaches to management may be indicated.

  View details for DOI 10.1016/j.amjcard.2005.04.024

  View details for Web of Science ID 000231255100022

  View details for PubMedID 16098316

• Left heart growth, function, and reintervention after balloon aortic valvuloplasty for neonatal aortic stenosis CIRCULATION McElhinney, D. B., Lock, J. E., Keane, J. F., Moran, A. M., Colan, S. D. 2005; 111 (4): 451-458

  Abstract

  Transcatheter balloon aortic valvuloplasty (BAVP) has become the first-line treatment for critical aortic stenosis (AS) in neonates. However, little is known about the growth and function of left heart structures or about patterns of reintervention on the left heart after neonatal BAVP.Between 1985 and 2002, 113 patients underwent neonatal BAVP at < or =60 days of age. There were 16 early deaths (14%), with a significant decrease from 1985 to 1993 (22%) to 1994 to 2002 (4%), and 6 patients had successful early conversion to a univentricular circulation. In the short term, the mean relative gradient reduction was 54+/-26%, and significant aortic regurgitation (AR) developed in 15% of patients. The 91 early survivors with a biventricular circulation were followed up for 6.3+/-5.3 years, during which time there was a steady increase in the frequency of significant AR. Freedom from moderate or severe AR was 65% at 5 years. In almost all patients with a baseline aortic annulus z score less than -1, the annulus diameter increased to within the normal range within 1 to 2 years. Similarly, left ventricular (LV) end-diastolic dimension z scores, which ranged from -5 to 7.5 before BAVP, normalized within 1 to 2 years in nearly all patients with a predilation z score less than -1. Among early survivors with a biventricular circulation, reintervention-free survival on the LV outflow tract was 65% at 1 year and 48% at 5 years, with younger age, higher pre- and post-BAVP gradients, and a larger balloon-annulus diameter ratio associated with decreased reintervention-free survival (P<0.01). Seventeen surgical interventions were performed on the aortic valve in 15 patients, including replacement in 7. Survival free from aortic valve replacement was 84% at 5 years.BAVP for AS during the first 60 days of life results in short-term relief of AS in the majority of patients. Among early survivors, initially small left heart structures may be associated with worse subacute outcomes but typically normalize within 1 year. Reintervention for residual/recurrent AS or iatrogenic AR is relatively common, particularly during the first year after BAVP, but aortic valve replacement during early childhood is seldom necessary.

  View details for DOI 10.1161/01.CIR.0000153809.88286.2E

  View details for Web of Science ID 000226692600014

  View details for PubMedID 15687133

• Recombinant human growth hormone treatment for dilated cardiomyopathy in children PEDIATRICS McElhinney, D. B., Colan, S. D., Moran, A. M., Wypij, D., Lin, M., Majzoub, J. A., Crawford, E. C., Bartlett, J. M., McGrath, E. A., Newburger, J. W. 2004; 114 (4): E452-E458

  Abstract

  Dilated cardiomyopathy (DCM) is one of the most common causes of heart failure among children and is often progressive despite maximal medical therapy. Heart failure is characterized by a number of neurohormonal abnormalities, including derangements in the growth hormone (GH)/insulin-like growth factor-1 (IGF-1) signaling axis. Decreased serum levels of GH, which acts on cardiac myocytes primarily through IGF-1, are associated with impaired myocardial growth and function, which can be improved with restoration of GH/IGF-1 homeostasis. In animal models and among human adults with heart failure attributable to DCM, treatment with GH results in acquisition of left ventricular (LV) mass and improved LV function, through a combination of mechanisms. We undertook this study to determine the effects of recombinant human GH on LV function and mass among children with stable LV dysfunction attributable to DCM.We performed a prospective, single-center, randomized, partially blinded, crossover trial among children 1 to 19 years of age with DCM and cardiac dysfunction of > or =6-month duration. After enrollment, patients were randomly assigned to receive treatment for 6 months with either conventional therapy (determined by the patient's primary cardiologist) plus recombinant human GH (0.025-0.04 mg/kg per day), administered as daily subcutaneous injections, or conventional therapy alone. Patients were then crossed over to the other treatment strategy for 6 months. The primary outcome measure was change in LV shortening fraction (SF). Other echocardiographic indices of LV function, somatic growth, and somatotropic/thyroid hormone levels were also monitored.Only 8 of an intended 15 patients were enrolled, because of a combination of factors. Two patients withdrew during the study as a result of declining LV function requiring transplantation. LV SF did not change significantly during GH treatment, although both LV SF and LV SF z score were higher 6 months after cessation of GH treatment than at baseline. LV ejection fraction increased during GH therapy to a degree that approached significance. Height and weight percentiles for age increased significantly during GH therapy and remained higher 6 months after treatment. Annualized height velocity during GH treatment (13.7 +/- 3.3 cm/year, >97th percentile for all patients) was significantly higher than that after GH discontinuation (3.2 +/- 3.5 cm/year). Serum levels of IGF-1 and IGF-binding protein-3 were significantly higher after 6 months of GH treatment and 6 months after discontinuation of GH treatment than at baseline. There were no adverse events related to GH treatment.In this prospective, single-center, randomized, partially blinded, crossover trial, recombinant human GH was administered to 8 pediatric patients with stable chronic heart failure secondary to DCM. Because of unanticipated difficulty enrolling eligible patients, the study was underpowered to detect changes in our primary outcome measure of the magnitude we projected. Nevertheless, we did observe several notable cardiovascular effects of GH treatment, including a trend toward improved LV ejection fraction during the course of GH treatment and significantly improved LV SF, SF z score, and LV end systolic stress z score 6 months after discontinuation of GH treatment (relative to baseline values). Given the fact that levels of IGF-1, the primary myocardial effector of GH signaling, remained significantly higher 6 months after GH treatment than at baseline, the improvement in LV functional indices 6 months after discontinuation of therapy may represent progression or perpetuation of a GH treatment effect. In addition to its cardiovascular effects, GH therapy was associated with significant acceleration of somatic growth. The benefits of GH were not associated with significant attributable side effects, although 2 patients developed progressive LV dysfunction during the study and underwent cardiac transplantation.

  View details for DOI 10.1542/peds.2004-0072

  View details for Web of Science ID 000224242200009

  View details for PubMedID 15466071

• Transcatheter device closure of congenital and postoperative residual ventricular septal defects CIRCULATION Knauth, A. L., Lock, J. E., Perry, S. B., McElhinney, D. B., Gauvreau, K., Landzberg, M. J., Rome, J. J., Hellenbrand, W. E., Ruiz, C. E., Jenkins, K. J. 2004; 110 (5): 501-507

  Abstract

  Our purpose was to describe a 13-year experience with patients undergoing transcatheter device closure of unrepaired congenital or postoperative residual ventricular septal defects (VSDs).Since 1989, 170 patients (median age, 3.9 years) have undergone catheterization for closure of 1 or more congenital (n=92) or postoperative (n=78) residual VSDs using successive generations of STARFlex-type devices. Outcomes included echocardiographic assessment of residual flow and device position, assessment of VSD shunt/severity, and adverse events. Among 168 patients in whom device implantation was performed, between 1 and 7 devices were placed per patient (median, 1), with multiple devices placed in 40%. There was a significant decrease in left-to-right shunting after device implantation (P<0.001) and significant improvement in VSD size/severity, and device position proved stable. Of 332 adverse events, 39 were related to the device and 261 were related to the catheterization; all but 5 occurred in the periprocedural period. At a median follow-up of 24 months (0 to 154 months), 14 patients had died and 18 had device(s) explanted.Congenital and postoperative VSD closure using STARFlex-type devices resulted in stable improvement in clinical status and decreased interventricular shunting. Although periprocedural events occurred frequently, late events caused by the device were rare. Transcatheter device closure is an effective management option for patients with complex muscular VSDs that are difficult to approach surgically and for postoperative residual VSDs.

  View details for DOI 10.1161/01.CIR.0000137116.12176.A6

  View details for Web of Science ID 000223055600006

  View details for PubMedID 15262841

• Hypoplastic left heart syndrome with intact or highly restrictive atrial Septum - Outcome after neonatal transcatheter atrial septostomy CIRCULATION Vlahos, A. P., Lock, J. E., McElhinney, D. B., van der Velde, M. E. 2004; 109 (19): 2326-2330

  Abstract

  Hypoplastic left heart syndrome (HLHS) with intact or very restrictive atrial septum is a highly lethal combination. We review our 13-year institutional experience treating this high-risk subgroup of patients with emergent catheter therapy.Infants with HLHS requiring catheter septostomy within the first 2 days of life were compared with a matched control group with adequate interatrial communication. Preoperative, early postoperative, and medium-term survival were evaluated. Earlier experience was compared with recent results to assess the effect of changes in catheterization and surgical and intensive care unit management strategies over the study period. From 1990 to 2002, 33 newborns with HLHS (11% of newborns with HLHS managed during this period) underwent urgent/semiurgent catheterization to create or enlarge an interatrial communication before surgical palliation. Preoperative and early postoperative mortality were high (48%) compared with control HLHS patients, regardless of prenatal diagnosis and despite successful catheter-based atrial septostomy with clinical stabilization. Mortality trended down during the later part of the study period. Those who survived the neonatal period had late survival, pulmonary artery pressure, and resistance similar to those of control subjects.Neonatal mortality in the subgroup of HLHS patients with intact or highly restrictive atrial septum remains high despite successful urgent septostomy. Persistently poor outcomes for these patients have prompted efforts at our center to develop techniques for fetal intervention for this condition, in the hope that prenatal relief of left atrial and pulmonary venous hypertension may promote normal pulmonary vascular and parenchymal development and improve both short- and long-term outcomes.

  View details for DOI 10.1161/01.CIR.0000128690.35860.C5

  View details for Web of Science ID 000221477800014

  View details for PubMedID 15136496

• Association between cardiac tumors and tuberous sclerosis in the fetus and neonate AMERICAN JOURNAL OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Margossian, R., Moon-Grady, A. J., Sallee, D., Goldmuntz, E., van der Velde, M. E., Silverman, N. H., Allan, L. D. 2003; 92 (4): 487-489

  Abstract

  A retrospective review was performed in 94 patients with > or =1 cardiac tumors seen on prenatal or neonatal echocardiography at 5 major referral centers. Tuberous sclerosis was present in 68 patients diagnosed with a cardiac tumor in utero or during the neonatal period, including 61 of 64 with multiple tumors.

  View details for DOI 10.1016/S0002-9149(03)00677-5

  View details for Web of Science ID 000184650200029

  View details for PubMedID 12914889

• Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome 47th Annual Scientific Session of the American-College-of-Cardiology Tworetzky, W., McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L., Silverman, N. H. LIPPINCOTT WILLIAMS & WILKINS. 2001: 1269–73

  Abstract

  Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05).Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

  View details for Web of Science ID 000167562200017

  View details for PubMedID 11238272

• Transesophageal echocardiographic predictors for successful transcatheter closure of defects within the oval fossa using the CardioSEAL((R)) septal occlusion device CARDIOLOGY IN THE YOUNG Momenah, T. S., McElhinney, D. B., Brook, M. M., Moore, P., Silverman, N. H. 2000; 10 (5): 510-518

  Abstract

  To define the utility of transesophageal echocardiography in predicting the likelihood of a successful procedure and residual shunting in patients undergoing transcatheter closure of defects within the oval fossa using the CardioSEAL device.Transesophageal echocardiography is used to monitor transcatheter closure of interatrial defects within the the oval fossa, but predictors of successful closure and residual shunting have yet to be determined.We reviewed transesophageal echocardiograms obtained from 26 consecutive patients undergoing attempted transcatheter closure of interatrial defects within the oval fossa between January, 1997 and May, 1998. Assessment of the atrial septum, the septal defect, and the rims of the oval fossa bordering the defect was performed in 3 planes: longitudinal, 4-chamber, and basal short-axis.Closure proved successful in 24 patients (92%). The defect was significantly larger, and the anterosuperior rim of the defect smaller, in the 2 patients in whom occlusion was not successful. Residual shunting 24 hrs after closure was detected in 14 patients. Significant predictors of leakage included smaller posterior and superior rims, a larger shunt prior to closure, and herniation of a one left atrial arm of the device into the right atrium. In all cases, the sites of leakage were the superior rim of the defect at the superior cavo-atrial junction, and the anterosuperior rim behind the aortic root. Herniation of a left atrial arm into the right atrium was seen in 7 patients (29%). In all, it was the anterosuperior arm which herniated Doppler color flow was suboptimal in detecting residual leaks, and was enhanced substantially with the use of contrast echocardiography.Transesophageal echocardiography allows excellent assessment of the oval fossa and deficiencies of its floor in all of their dimensions. It is an important tool for guiding the deployment of the occlusion device in patients undergoing attempted transcatheter closure of defects within the fossa. Contrast echocardiography should be used for optimal detection of residual shunting.

  View details for Web of Science ID 000089640900013

  View details for PubMedID 11049127

• Incidence and implications of systemic to pulmonary collaterals after bidirectional cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Petrossian, E., Hanley, F. L., Moore, P. 2000; 69 (4): 1222-1228

  Abstract

  Systemic to pulmonary arterial collaterals often develop after bidirectional cavopulmonary anastomosis (BCPA). It has been proposed that such collaterals may be related to perioperative outcome and duration of effusions after the modified Fontan procedure. However, the incidence and significance of collaterals after BCPA remain uncertain.To evaluate risk factors for, and significance of, such collaterals, we reviewed angiographic and clinical data for all 76 patients who underwent BCPA between January 1990 and June 1996 and had follow-up catheterization during or before 1997.The median age at BCPA was 10 months, and the median duration from BCPA to follow-up catheterization was 18 months. Arterial collaterals were detected on follow-up catheterization in 45 patients (59%). Factors associated with collateral development included a prior right-sided systemic-to-pulmonary arterial shunt, lower pre-BCPA end-diastolic ventricular pressure and pulmonary vascular resistance, and use and duration of cardiopulmonary bypass during the BCPA operation. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Forty-three patients have undergone extracardiac conduit Fontan, with 1 early and 1 late death. Collaterals were present in 22 of these patients, 7 of whom underwent pre-Fontan embolization. The duration from BCPA to Fontan was longer in patients with collaterals, but these patients were not more likely to have prolonged effusions than those without, and the duration of tube thoracostomy was significantly shorter in patients with collaterals. Embolization of collaterals did not affect the duration of effusions.Systemic-to-pulmonary arterial collaterals are common after BCPA. In contrast to prior reports, collaterals were not associated with a higher incidence of prolonged effusions after the Fontan procedure in our experience, and did not correlate with poor outcome.

  View details for Web of Science ID 000086808500059

  View details for PubMedID 10800823

• Issues and outcomes in the management of supravalvar aortic stenosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Tworetzky, W., Silverman, N. H., Hanley, F. L. 2000; 69 (2): 562-567

  Abstract

  Supravalvar stenosis of the aorta is an uncommon congenital cardiac anomaly that involves not only the supravalvar aorta but the entire aortic root. Despite considerable attention to the importance of maintaining the integrity of the aortic root during supravalvar reconstruction, there has been little focus on the management of other components of the aortic root and left ventricular outflow tract, including the aortic valve, subvalvar region, and coronary arteries.We reviewed the records of 36 consecutive patients with supravalvar aortic stenosis who underwent repair from 1992-1998 (median age, 4 years). Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. Associated anomalies of the aortic root and adjacent structures were present in 23 patients. The median pressure gradient across the left ventricular outflow tract was 70 mm Hg. Supravalvar stenosis was relieved by extended aortoplasty with a Y-shaped patch in 18 patients, resection of the stenotic segment of ascending aorta at the sinotubular junction with end-to-end anastomosis of the ascending aorta in 7, the Ross procedure in 4, and other techniques in 7. Additional procedures included aortic valvuloplasty in 10 patients, resection of subvalvar stenosis in 11, and procedures on the coronary arteries in 2.There was 1 perioperative death, and no reoperations or other significant complications. During follow-up (median 33 months), there were no deaths and 3 reoperations for replacement of the aortic valve with a pulmonary autograft (n = 1) or mechanical prosthesis (n = 2). The median pressure gradient across the left ventricular outflow tract was 10 mm Hg.In patients with supravalvar aortic stenosis, abnormalities of the aortic valve, subaortic region, and coronary arteries are frequently present as well. Management of these issues is as critical to the long-term outcome of these patients as reconstruction of the supravalvar aorta. Aggressive valvuloplasty may help decrease the incidence of late aortic valve replacement, whereas the Ross procedure may be a preferable approach in some patients with complex outflow tract obstruction.

  View details for Web of Science ID 000085382200061

  View details for PubMedID 10735699

• Congenital obstructive lesions of the right aortic arch ANNALS OF THORACIC SURGERY McElhinney, D. B., Tworetzky, W., Hanley, F. L., Rudolph, A. M. 1999; 67 (4): 1194-1202

  Abstract

  In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.

  View details for Web of Science ID 000080115300078

  View details for PubMedID 10320289

• Multiple ventricular septal defects: How and when should they be repaired? 78th Annual Meeting of the American-Association-for-Thoracic-Surgery Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. MOSBY-ELSEVIER. 1999: 134–39

  Abstract

  Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined.From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy.One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects.In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.

  View details for Web of Science ID 000077898200027

  View details for PubMedID 9869767

• Echocardiographic diagnosis alone for the complete repair of major congenital heart defects JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Brook, M. M., Reddy, V. M., Hanley, F. L., Silverman, N. H. 1999; 33 (1): 228-233

  Abstract

  The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

  View details for Web of Science ID 000078088300036

  View details for PubMedID 9935035

• Intramyocardial hematoma causing cardiac tamponade after repair of Ebstein malformation: Erroneous echocardiographic diagnosis as intracavitary thrombus JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Momenah, T. S., McElhinney, D. B., Brook, M. M., Teitel, D. F., Hanley, F. L., Silverman, N. H. 1998; 11 (11): 1087-1089

  Abstract

  Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the right atrium to the atrialized right ventricle. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention.

  View details for Web of Science ID 000077038900014

  View details for PubMedID 9812104

• Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants < 6 months of age AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1998; 81 (2): 195-201

  Abstract

  The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

  View details for Web of Science ID 000071617500015

  View details for PubMedID 9591904

• An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it? 22nd Annual Meeting of the Western-Thoracic-Surgical-Association Reddy, V. M., McElhinney, D. B., Moore, P., Bristow, J., Haas, G. S., Hanley, F. L. GREENWICH MEDICAL MEDIA LTD. 1997: 284–93

  View details for Web of Science ID A1997XV28900010