Lynn Peng

All Publications

• 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

• 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

• 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

• Acute Hemodynamics in the Fontan Circulation: Open-Label Study of Vasopressin. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Adamson, G. T., Yu, J., Ramamoorthy, C., Peng, L. F., Taylor, A., Lennig, M., Schmidt, A. R., Feinstein, J. A., Navaratnam, M. 2023

  Abstract

  OBJECTIVE: To describe the acute hemodynamic effect of vasopressin on the Fontan circulation, including systemic and pulmonary pressures and resistances, left atrial pressure, and cardiac index.DESIGN: Prospective, open-label, nonrandomized study (NCT04463394).SETTING: Cardiac catheterization laboratory at Lucile Packard Children's Hospital, Stanford.PATIENTS: Patients 3-50 years old with a Fontan circulation who were referred to the cardiac catheterization laboratory for hemodynamic assessment and/or intervention.INTERVENTIONS: A 0.03 U/kg IV (maximum dose 1 unit) bolus of vasopressin was administered over 5 minutes, followed by a maintenance infusion of 0.3 mU/kg/min (maximum dose 0.03 U/min).MEASUREMENTS AND MAIN RESULTS: Comprehensive cardiac catheterization measurements before and after vasopressin administration. Measurements included pulmonary artery, atrial, and systemic arterial pressures, oxygen saturations, and systemic and pulmonary flows and resistances. There were 28 patients studied. Median age was 13.5 (9.1, 17) years, and 16 (57%) patients had a single or dominant right ventricle. Following vasopressin administration, systolic blood pressure and systemic vascular resistance (SVR) increased by 17.5 (13.0, 22.8) mm Hg (Z value -4.6, p < 0.001) and 3.8 (1.8, 7.5) Wood Units (Z value -4.6, p < 0.001), respectively. The pulmonary vascular resistance (PVR) decreased by 0.4±0.4 WU (t statistic 6.2, p < 0.001), and the left atrial pressure increased by 1.0 (0.0, 2.0) mm Hg (Z value -3.5, p < 0.001). The PVR:SVR decreased by 0.04±0.03 (t statistic 8.1, p < 0.001). Neither the pulmonary artery pressure (median difference 0.0 [-1.0, 1.0], Z value -0.4, p = 0.69) nor cardiac index (0.1±0.3, t statistic -1.4, p = 0.18) changed significantly. There were no adverse events.CONCLUSIONS: In Fontan patients undergoing cardiac catheterization, vasopressin administration resulted in a significant increase in systolic blood pressure, SVR, and left atrial pressure, decrease in PVR, and no change in cardiac index or pulmonary artery pressure. These findings suggest that in Fontan patients vasopressin may be an option for treating systemic hypotension during sedation or general anesthesia.

  View details for DOI 10.1097/PCC.0000000000003326

  View details for PubMedID 37462430

• First described mitral clip in an adult extracardiac Fontan patient: a case report. European heart journal. Case reports Haeffele, C. L., Lui, G. K., Peng, L., Chan, F., Sharma, R. P. 2023; 7 (1): ytac479

  Abstract

  The use of transcatheter edge-to-edge repair (TEER) in patients with advanced heart failure has been shown to reduce hospitalizations and increase survival. As patients with Fontan circulations grow older, a significant proportion of them will develop severe atrioventricular (AV) valve regurgitation in the systemic ventricle. Conventional surgical repair and transplant carry high mortality risk for the adult Fontan patient with progressive heart failure.A 51-year-old female extracardiac Fontan patient developed severe AV valve regurgitation and progressive functional decline. Based on her operative risk for conventional surgical intervention or transplant, TEER using the Abbott MitraClip device was performed. The degree of mitral regurgitation was decreased from severe to moderate regurgitation.This is the first known case describing the use of a successful TEER in an adult patient with an extracardiac Fontan. Given the increasing numbers of patients surviving into adulthood with a Fontan circulation, transcatheter interventions may provide an alternative treatment option to conventional surgeries and medical therapies.

  View details for DOI 10.1093/ehjcr/ytac479

  View details for PubMedID 36733686

  View details for PubMedCentralID PMC9887705

• The development and efficacy of a paediatric cardiology fellowship online preparatory course. Cardiology in the young Motonaga, K. S., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J. A., Silverman, N. H., Hanley, F. L., Axelrod, D. M., Krawczeski, C. D., Arunamata, A., Kwiatkowski, D. M., Ceresnak, S. R. 2022: 1-6

  Abstract

  BACKGROUND: The transition from residency to paediatric cardiology fellowship is challenging due to the new knowledge and technical skills required. Online learning can be an effective didactic modality that can be widely accessed by trainees. We sought to evaluate the effectiveness of a paediatric cardiology Fellowship Online Preparatory Course prior to the start of fellowship.METHODS: The Online Preparatory Course contained 18 online learning modules covering basic concepts in anatomy, auscultation, echocardiography, catheterisation, cardiovascular intensive care, electrophysiology, pulmonary hypertension, heart failure, and cardiac surgery. Each online learning module included an instructional video with pre-and post-video tests. Participants completed pre- and post-Online Preparatory Course knowledge-based exams and surveys. Pre- and post-Online Preparatory Course survey and knowledge-based examination results were compared via Wilcoxon sign and paired t-tests.RESULTS: 151 incoming paediatric cardiology fellows from programmes across the USA participated in the 3 months prior to starting fellowship training between 2017 and 2019. There was significant improvement between pre- and post-video test scores for all 18 online learning modules. There was also significant improvement between pre- and post-Online Preparatory Course exam scores (PRE 43.6 ± 11% versus POST 60.3 ± 10%, p < 0.001). Comparing pre- and post-Online Preparatory Course surveys, there was a statistically significant improvement in the participants' comfort level in 35 of 36 (97%) assessment areas. Nearly all participants (98%) agreed or strongly agreed that the Online Preparatory Course was a valuable learning experience and helped alleviate some anxieties (77% agreed or strongly agreed) related to starting fellowship.CONCLUSION: An Online Preparatory Course prior to starting fellowship can provide a foundation of knowledge, decrease anxiety, and serve as an effective educational springboard for paediatric cardiology fellows.

  View details for DOI 10.1017/S1047951122003626

  View details for PubMedID 36440543

• 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

• 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

• 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

• 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

• Maternal Hyperoxygenation Testing in Fetuses with Hypoplastic Left-Heart Syndrome: Association with Postnatal Atrial Septal Restriction. Fetal diagnosis and therapy Mardy, C., Kaplinski, M., Peng, L., Blumenfeld, Y. J., Kwiatkowski, D. M., Tacy, T. A., Maskatia, S. A. 2021: 1-12

  Abstract

  INTRODUCTION: In fetuses with hypoplastic left-heart syndrome (HLHS), maternal hyperoxygenation (MHO) may aid risk stratification. We hypothesized that pulmonary vein (Pvein) velocity time integral (VTI) change with MHO would more reliably identify neonates who undergo emergent atrial septoplasty (EAS) than changes in pulmonary arterial pulsatility index (PA PI).METHODS: Fetuses with HLHS who underwent MHO testing at our institution between 2014 and 2019 were identified. Data were reviewed in a blinded, retrospective manner. Pvein VTI ratio (prograde:retrograde) was calculated. The primary outcome was neonatal EAS.RESULTS: Twenty-seven HLHS fetuses underwent MHO, and 5 (19%) underwent EAS. Without MHO, a Pvein VTI ratio <3 conferred 60% sensitivity and 100% specificity for EAS. With MHO, a Pvein VTI ratio <6.5 conferred 100% sensitivity and specificity. For an intermediate group of fetuses with a baseline Pvein VTI ratio 3-7, the ratio decrease with MHO conferred 100% sensitivity and specificity. Compared to the Pvein VTI ratio, PA PI was less accurate in identifying EAS neonates.DISCUSSION/CONCLUSION: Addition of MHO appears to improve the diagnostic ability of the Pvein VTI ratio to identify HLHS fetuses who undergo EAS. The Pvein VTI ratio change may more accurately identify fetuses who undergo EAS than change in PA PI and has less interobserver variability.

  View details for DOI 10.1159/000519322

  View details for PubMedID 34673647

• 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

• Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome. Pediatric cardiology Balasubramanian, S., Smith, S. N., Srinivasan, P., Tacy, T. A., Hanley, F. L., Chen, S., Wright, G. E., Peng, L. F., Punn, R. 2021

  Abstract

  Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71±4.4%, 69±4.5% and 66±4.7% at 1, 2 and 5years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7±9.3% to 31.1±8.3% (p<0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9±1.3 to -9.7±1.3 (p<0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC>35% and TAPSE Z-score>-5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p<0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.

  View details for DOI 10.1007/s00246-021-02624-y

  View details for PubMedID 33987707

• 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

• 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

• 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

• Adverse Events Associated with Cardiac Catheterization in Children Supported with Ventricular Assist Devices. ASAIO journal (American Society for Artificial Internal Organs : 1992) Power, A., Navaratnam, M., Murray, J. M., Peng, L. F., Rosenthal, D. N., Dykes, J. C., Yarlagadda, V. V., Maeda, K., Almond, C. S., Chen, S. 2021

  Abstract

  Children on ventricular assist device (VAD) support can present several unique challenges, including small patient size, univentricular or biventricular congenital heart disease (1V- or 2V-CHD) and need for biventricular VAD (BiVAD) support. While cardiac catheterization can provide valuable information, it is an invasive procedure with inherent risks. We sought to evaluate the safety of catheterization in pediatric patients on VAD support. We performed a retrospective review of patients on VAD support who underwent catheterization at Lucile Packard Children's Hospital between January 1, 2014 and September 1, 2019. Using definitions adapted from Pedimacs, adverse events (AEs) after catheterization were identified, including arrhythmia; major bleeding or acute kidney injury within 24 hours; respiratory failure persisting at 24 hours; and stroke, pericardial effusion, device malfunction, bacteremia or death within 7 days. AEs were categorized as related or unrelated to catheterization. Sixty procedures were performed on 39 patients. Underlying diagnoses were dilated cardiomyopathy (48%), 1V-CHD (35%), 2V-CHD (8%), and other (8%). Devices were implantable continuous flow (72%), paracorporeal pulsatile (18%) and paracorporeal continuous flow (10%). Catheterizations were performed on patients in the ICU (60%), on inotropic support (42%), with deteriorating clinical status (37%) and on BiVAD support (12%). There were 9 AEs possibly related to catheterization including 6 episodes of respiratory failure, 2 major bleeding events, and 1 procedural arrhythmia. AE occurrence was associated with ICU status (P = 0.01), BiVAD support (P = 0.04) and procedural indication to evaluate worsening clinical status (P = 0.04). Despite high medical acuity, catheterization can be performed with an acceptable AE profile in children on VAD support.

  View details for DOI 10.1097/MAT.0000000000001627

  View details for PubMedID 34967779

• Adverse Events Following Cardiac Catheterization among Infants, Children, and Young Adults on Ventricular Assist Device Support Power, A., Murray, J. M., Peng, L. F., Rosenthal, D. N., Dykes, J. C., Shui, A. A., Yarlagadda, V. V., Navaratnam, M., Maeda, K., Almond, C. S., Chen, S. ELSEVIER SCIENCE INC. 2020: S465–S466

  View details for Web of Science ID 000522637203169

• 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

• Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019; 93 (4): 660–63

  View details for DOI 10.1002/ccd.28073

  View details for Web of Science ID 000461016400030

• 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

• 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

• Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019

  Abstract

  Clinically significant unilateral pulmonary arteriovenous malformations (PAVM) can develop in patients with a Fontan circulation when there is unbalanced distribution of hepatic venous (HV) blood flow to the lungs. There are reported surgical and transcatheter techniques to treat PAVMs by rerouting HV return, with promising short-term results. We report a case of a novel, technically simple transcatheter approach to redirect HV blood flow in an adult Fontan patient with polysplenia syndrome and severe unilateral PAVMs. Our patient had a two-stage procedure, the first to redirect all HV blood flow to the affected lung with a single covered stent, and a second to confirm resolution of PAVMs and to reintroduce HV effluent to the unaffected lung. At 10-month follow-up, her oxygen saturations had increased from 75% to 93% with a marked improvement in her functional status.

  View details for PubMedID 30629315

• 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

• Surgical unroofing of hemodynamically significant myocardial bridges in a pediatric population Maeda, K., Schnittger, I., Murphy, D. J., Tremmel, J. A., Boyd, J. H., Peng, L., Okada, K., Pargaonkar, V. S., Hanley, F. L., Mitchell, R., Rogers, I. S. MOSBY-ELSEVIER. 2018: 1618–26

  View details for DOI 10.1016/j.jtcvs.2018.01.081

  View details for Web of Science ID 000445221200079

• 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

• Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung ANNALS OF THORACIC SURGERY Mainwaring, R. D., Rosenblatt, T. R., Patrick, W. L., Ma, M., Peng, L., Hanley, F. L. 2018; 106 (2): 568–74

  View details for DOI 10.1016/j.athoracsur.2018.03.041

  View details for Web of Science ID 000439475700056

• Surgical unroofing of hemodynamically significant myocardial bridges in a pediatric population. The Journal of thoracic and cardiovascular surgery Maeda, K., Schnittger, I., Murphy, D. J., Tremmel, J. A., Boyd, J. H., Peng, L., Okada, K., Pargaonkar, V. S., Hanley, F. L., Mitchell, R. S., Rogers, I. S. 2018

  Abstract

  BACKGROUND: Although myocardial bridges (MBs) are traditionally regarded as incidental findings, it has been reported that adult patients with symptomatic MBs refractory to medical therapy benefit from unroofing. However, there is limited literature in the pediatric population. The aim of our study was to evaluate the indications and outcomes for unroofing in pediatric patients.METHODS: We retrospectively reviewed all pediatric patients with MB in our institution who underwent surgical relief. Clinical characteristics, relevant diagnostic data, intraoperative findings, and postoperative outcomes were evaluated.RESULTS: Between 2012 and 2016, 14 pediatric patients underwent surgical unroofing of left anterior descending artery MBs. Thirteen patients had anginal symptoms refractory to medical therapy, and 1 patient was asymptomatic until experiencing aborted sudden cardiac arrest during exercise. Thirteen patients underwent exercise stress echocardiography, all of which showed mid-septal dys-synergy. Coronary computed tomography imaging confirmed the presence of MBs in all patients. Intravascular ultrasound imaging confirmed the length of MBs: 28.2±16.3mm, halo thickness: 0.59±0.24mm, and compression of left anterior descending artery at resting heart rate: 33.0±11.6%. Invasive hemodynamic assessment with dobutamine confirmed the physiologic significance of the MBs with diastolic fractional flow reserve: 0.59±0.13. Unroofing was performed with the patient under cardiopulmonary bypass (CPB) in the initial 9 cases and without CPB in the subsequent 5 cases. All patients were discharged without complications. The 13 symptomatic patients reported resolution of symptoms on follow-up, and improvement in symptoms and quality of life was documented using the Seattle Angina Questionnaire version 7.CONCLUSIONS: Unroofing of MBs can be safely performed in pediatric patients, with or without use of CPB. In symptomatic patients, unroofing can provide relief of symptoms refractory to medical therapy.

  View details for PubMedID 30005887

• An unexpected case of post-operative superior caval vein syndrome. Cardiology in the young Peng, L., Wise-Faberowski, L. 2018; 28 (6): 879–81

  Abstract

  Superior caval vein obstruction in children after congenital heart surgery has been more associated with thrombosis formation as result of single-ventricle palliation, infection, indwelling devices/catheters, or external compression. Many of these patients will present to the cardiac catheterisation laboratory for evaluation and possible intervention. We present an unusual case of superior caval vein obstruction in a patient after Tetralogy of Fallot repair.

  View details for PubMedID 29697045

• An unexpected case of post-operative superior caval vein syndrome CARDIOLOGY IN THE YOUNG Peng, L., Wise-Faberowski, L. 2018; 28 (6): 879–81

  View details for DOI 10.1017/S1047951118000227

  View details for Web of Science ID 000434250000016

• Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung. The Annals of thoracic surgery Mainwaring, R. D., Rosenblatt, T. R., Patrick, W. L., Ma, M., Peng, L., Hanley, F. L. 2018

  Abstract

  BACKGROUND: There are patients born with pulmonary atresia and a ductus arteriosus or hemi-truncus to one lung and major aortopulmonary collateral arteries (MAPCAs) to the contralateral lung. The purpose of this study was to review our surgical results for this relatively rare subset of patients.METHODS: This was a retrospective review of 35 patients with ductus/hemi-truncus in association with pulmonary atresia with ventricular septal defect and MAPCAs. Our surgical algorithm is bifurcated into two strategies: 1) patients with "favorable" MAPCAs are candidates for single stage complete repair, and 2) patients with "unfavorable" MAPCAs undergo a staged approach. The median age at surgery was 3 months (range 1-7 months), and the mean number of MAPCAs was 3.1 ± 1.3.RESULTS: Twenty patients underwent a single stage complete repair. All 20 of these patients are alive with a right ventricle to aortic pressure ratio of 0.34 ± 0.07. The distribution of pulmonary blood flow by lung perfusion scan was 44% to the right and 56% to the left lung. Fifteen patients underwent a staged approach, including unifocalization of the MAPCAs to a central shunt. There were two interim deaths. Twelve patients have undergone complete repair with a right ventricle to aortic pressure ratio of 0.38 ± 0.07. There was one late death after complete repair. The distribution of pulmonary blood flow was 39% to the right and 61% to the left lung.CONCLUSIONS: The data demonstrate that 32 of 35 patients with ductus/hemi-truncus and MAPCAs ultimately achieved complete repair with relatively low pulmonary artery pressures.

  View details for PubMedID 29684371

• 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

• 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

• THE DEVELOPMENT AND EFFICACY OF A PEDIATRIC CARDIOLOGY FELLOWSHIP ONLINE PREPARATORY COURSE Motonaga, K., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J., Silverman, N., Hanley, F., Axelrod, D., Krawczeski, C., Ceresnak, S. ELSEVIER SCIENCE INC. 2018: 2622

  View details for DOI 10.1016/S0735-1097(18)33163-2

  View details for Web of Science ID 000429659705072

• 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

• 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

• 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

• Hemodynamic Effects of Phenylephrine, Vasopressin, and Epinephrine in Children With Pulmonary Hypertension: A Pilot Study PEDIATRIC CRITICAL CARE MEDICINE Siehr, S. L., Feinstein, J. A., Yang, W., Peng, L. F., Ogawa, M. T., Ramamoorthy, C. 2016; 17 (5): 428-437

  Abstract

  During a pulmonary hypertensive crisis, the marked increase in pulmonary vascular resistance can result in acute right ventricular failure and death. Currently, there are no therapeutic guidelines for managing an acute crisis. This pilot study examined the hemodynamic effects of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertension.In this prospective, open-label, nonrandomized pilot study, we enrolled pediatric patients previously diagnosed with pulmonary hypertensive who were scheduled electively for cardiac catheterization. Primary outcome was a change in the ratio of pulmonary-to-systemic vascular resistance. Baseline hemodynamic data were collected before and after the study drug was administered.Eleven of 15 participants were women, median age was 9.2 years (range, 1.7-14.9 yr), and median weight was 26.8 kg (range, 8.5-55.2 kg). Baseline mean pulmonary artery pressure was 49 ± 19 mm Hg, and mean indexed pulmonary vascular resistance was 10 ± 5.4 Wood units. Etiology of pulmonary hypertensive varied, and all were on systemic pulmonary hypertensive medications.Patients 1-5 received phenylephrine 1 μg/kg; patients 6-10 received arginine vasopressin 0.03 U/kg; and patients 11-15 received epinephrine 1 μg/kg. Hemodynamics was measured continuously for up to 10 minutes following study drug administration.After study drug administration, the ratio of pulmonary-to-systemic vascular resistance decreased in three of five patients receiving phenylephrine, five of five patients receiving arginine vasopressin, and three of five patients receiving epinephrine. Although all three medications resulted in an increase in aortic pressure, only arginine vasopressin consistently resulted in a decrease in the ratio of systolic pulmonary artery-to-aortic pressure.This prospective pilot study of phenylephrine, arginine vasopressin, and epinephrine in pediatric patients with pulmonary hypertensive showed an increase in aortic pressure with all drugs although only vasopressin resulted in a consistent decrease in the ratio of pulmonary-to-systemic vascular resistance. Studies with more subjects are warranted to define optimal dosing strategies of these medications in an acute pulmonary hypertensive crisis.

  View details for DOI 10.1097/PCC.0000000000000716

  View details for PubMedID 27144689

• Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries ANNALS OF THORACIC SURGERY Carrillo, S. A., Mainwaring, R. D., Patrick, W. L., Bauser-Heaton, H. D., Peng, L., Reddy, V. M., Hanley, F. L. 2015; 100 (2): 606-614

  Abstract

  One anatomic variant of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is characterized by the absence of intrapericardial pulmonary arteries. This anatomy obviates the possibility of incorporating the pulmonary arteries for reconstruction or palliative procedures. The purpose of this study was to evaluate the surgical results in patients undergoing repair of PA/VSD/MAPCAs with absent pulmonary arteries.This was a retrospective review of 35 patients who underwent surgical repair of PA/VSD/MAPCAs with absent pulmonary arteries between 2007 and 2014. The median age at the time of surgery was 3.4 months, and the median weight was 4.9 kg. All patients underwent unifocalization of MAPCAs, with an average of 3.5 ± 1.4 MAPCAs per patient.Twenty-eight of the 35 patients (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, VSD closure, and right ventricle to pulmonary artery conduit. After complete repair, the average right ventricular to aortic pressure ratio was 0.33 ± 0.07. There were no deaths in this subgroup. Seven patients (20%) were not deemed suitable candidates for VSD closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative death and 1 interim death. Three patients have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment.The majority of patients with PA/VSD/MAPCAs and absent pulmonary arteries can undergo complete single-stage repair with satisfactory postoperative hemodynamics. These results suggest that unifocalization of MAPCAs can provide a reasonable pulmonary vascular bed in the absence of intrapericardial pulmonary arteries.

  View details for DOI 10.1016/j.athoracsur.2015.03.110

  View details for PubMedID 26138766

• Outcomes Following Cardiac Catheterization After Congenital Heart Surgery CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Siehr, S. L., Martin, M. H., Axelrod, D., Efron, B., Peng, L., Roth, S. J., Perry, S., Shin, A. Y. 2014; 84 (4): 622-628

  Abstract

  Describe outcomes following unplanned cardiac catheterization after congenital heart surgery.Utility of cardiac catheterization following congenital heart surgery is relatively understudied.Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution.Between October 2004 and April 2011, 120 patients underwent 150 unplanned postoperative cardiac catheterizations. Median day of catheterization was postoperative day 20 (range 1-269 days). Survival 30 days postcatheterization was 85%; overall survival to hospital discharge was 72%. Indications for catheterization: 63 for hemodynamic evaluation, 46 for likely intervention, and 41 for assessment of surgical repair. Of the 150 hemodynamic/interventional catheterizations, 103 (69%) were associated with a change in clinical management: 59 trans-catheter interventions, 22 re-operations, 11 changes in medication, six changes in surgical plan, and five withdrawals of support. Complications included hemorrhage in two patients, supraventricular tachycardia in two patients, and transient complete heart block requiring cardiopulmonary resuscitation in one patient.Cardiac catheterization following congenital heart surgery may enable important diagnostic and therapeutic changes in clinical and surgical management. Complications were rare.

  View details for DOI 10.1002/ccd.25490

  View details for Web of Science ID 000342826900018

• Outcomes following cardiac catheterization after congenital heart surgery. Catheterization and cardiovascular interventions Siehr, S. L., Martin, M. H., Axelrod, D., Efron, B., Peng, L., Roth, S. J., Perry, S., Shin, A. Y. 2014; 84 (4): 622-628

  Abstract

  Describe outcomes following unplanned cardiac catheterization after congenital heart surgery.Utility of cardiac catheterization following congenital heart surgery is relatively understudied.Retrospective study examining demographics, indications, and outcomes of unplanned cardiac catheterization after congenital heart surgery at a single institution.Between October 2004 and April 2011, 120 patients underwent 150 unplanned postoperative cardiac catheterizations. Median day of catheterization was postoperative day 20 (range 1-269 days). Survival 30 days postcatheterization was 85%; overall survival to hospital discharge was 72%. Indications for catheterization: 63 for hemodynamic evaluation, 46 for likely intervention, and 41 for assessment of surgical repair. Of the 150 hemodynamic/interventional catheterizations, 103 (69%) were associated with a change in clinical management: 59 trans-catheter interventions, 22 re-operations, 11 changes in medication, six changes in surgical plan, and five withdrawals of support. Complications included hemorrhage in two patients, supraventricular tachycardia in two patients, and transient complete heart block requiring cardiopulmonary resuscitation in one patient.Cardiac catheterization following congenital heart surgery may enable important diagnostic and therapeutic changes in clinical and surgical management. Complications were rare.

  View details for DOI 10.1002/ccd.25490

  View details for PubMedID 24659225

• Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance. journal of thoracic and cardiovascular surgery Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565

  Abstract

  Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.1±4.2 vs 5.6±4.5 days, respectively; P=.57).Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

  View details for DOI 10.1016/j.jtcvs.2014.01.017

  View details for PubMedID 24534681

• Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565

  Abstract

  Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.1±4.2 vs 5.6±4.5 days, respectively; P=.57).Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

  View details for DOI 10.1016/j.jtcvs.2014.01.017

  View details for Web of Science ID 000342896200077

• Melody (R) Pulmonary Valve Bacterial Endocarditis: Experience in Four Pediatric Patients and a Review of the Literature CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Villafane, J., Baker, G. H., Austin, E. H., Miller, S., Peng, L., Beekman, R. 2014; 84 (2): 212-218

  Abstract

  The objectives of this manuscript are two-fold: (a) to describe the clinical characteristics and management of four pediatric patients with bacterial endocarditis (BE) after Melody pulmonary valve implantation (MPVI); and (b) to review the literature regarding Melody pulmonary valve endocarditis.There are several reports of BE following MPVI. The clinical course, BE management and outcome remain poorly defined.This is a multi-center report of four pediatric patients with repaired tetralogy of Fallot (TOF) and BE after MPVI. Clinical presentation, echocardiogram findings, infecting organism, BE management, and follow-up assessment are described. We review available literature on Melody pulmonary valve endocarditis and discuss the prognosis and challenges in the management of these patients.Of our four BE patients, two had documented vegetations and three showed worsening pulmonary stenosis. All patients remain asymptomatic after medical treatment (4) and surgical prosthesis replacement (3) at follow-up of 17 to 40 months. Analysis of published data shows that over half of patients undergo bioprosthesis explantation and that there is a 13% overall mortality. The most common BE pathogens are the Staphylococcus and Streptococcus species.Our case series of four pediatric patients with repaired TOF confirms a risk for BE after MPVI. A high index of suspicion for BE should be observed after MPVI. All patients should be advised to follow lifelong BE prophylaxis after MPVI. In case of BE, surgery should be considered for valve dysfunction or no clinical improvement in spite of medical treatment.

  View details for DOI 10.1002/ccd.25375

  View details for Web of Science ID 000340554200009

  View details for PubMedID 24403185

• Transcatheter device closure of a congenital aortic-left atrial tunnel. Congenital heart disease Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-6

  Abstract

  Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

  View details for DOI 10.1111/chd.12059

  View details for PubMedID 23601836

• Transcatheter Device Closure of a Congenital Aortic-Left Atrial Tunnel CONGENITAL HEART DISEASE Sun, H. Y., Buccola, K. J., Punn, R., Silverman, N. H., Peng, L. F., Perry, S. B., Balasubramanian, S. 2014; 9 (1): E23-E26

  Abstract

  Rare cases of aortic-left atrial tunnel exist in the literature. This case report highlights the echocardiographic characterization of this vascular anomaly and provides the first description of an aortic-left atrial tunnel closed by interventional cardiac catheterization in a pediatric patient.

  View details for DOI 10.1111/chd.12059

  View details for Web of Science ID 000329916300010

  View details for PubMedID 23601836

• Hemodynamic Assessment After Complete Repair of Pulmonary Atresia With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Peng, L., Kuan, C., Palmon, M., Hanley, F. L. 2013; 95 (4): 1397-1402

  Abstract

  Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5±1.2 years, and the average interval between complete repair and conduit change was 4.5±1.1 years.The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70±22 mm Hg and pulmonary artery pressure of 38±14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34±8 mm Hg, similar to 36±9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36±0.07 and 0.39±0.09, respectively.The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.

  View details for DOI 10.1016/j.athoracsur.2012.12.066

  View details for PubMedID 23453744

• Evaluation of Pediatric Near-Infrared Cerebral Oximeter for Cardiac Disease Late-Breaking Clinical Trial Abstract Session on Congenital Heart Surgery at the 48th Annual Meeting of the Society-of-Thoracic-Surgeons / Surgical Motion Picture Session Kreeger, R. N., Ramamoorthy, C., Nicolson, S. C., Ames, W. A., Hirsch, R., Peng, L. F., Glatz, A. C., Hill, K. D., Hoffman, J., Tomasson, J., Kurth, C. D. ELSEVIER SCIENCE INC. 2012: 1527–33

  Abstract

  Cerebral hypoxia-ischemia remains a complication in children with congenital heart disease. Near-infrared spectroscopy can be utilized at the bedside to detect cerebral hypoxia-ischemia. This study aimed to calibrate and validate an advanced technology near-infrared cerebral oximeter for use in children with congenital heart disease.After institutional review board approval and parental consent, 100 children less than 12 years and less than 40 kg were enrolled. Phase I (calibration) measured arterial and jugular venous saturation (SaO(2), SjO(2)) by co-oximetry simultaneously with device signals to calibrate an algorithm to determine regional cerebral saturation against a weighted average cerebral saturation (0.7 SjO(2) + 0.3 SaO(2)). Phase II (validation) evaluated regional cerebral saturation from the algorithm against the weighted average cerebral saturation by correlation, bias, precision, and A(Root Mean Square) assessed by linear regression and Bland-Altman analysis.Of 100 patients, 86 were evaluable consisting of 7 neonates, 44 infants, and 35 children of whom 55% were female, 79% Caucasian, and 41% with cyanotic disease. The SaO(2) and regional cerebral saturation ranged from 34% to 100% and 34% to 91%, respectively. There were no significant differences in subject characteristics between phases. For the entire cohort, A(RMS), bias, precision, and correlation coefficient were 5.4%, 0.5%, 5.39%, and 0.88, respectively. Age, skin color, and hematocrit did not affect these values.This cerebral oximeter accurately measures the absolute value of cerebral saturation in children over a wide range of oxygenation and subject characteristics, offering advantages in assessment of cerebral hypoxia-ischemia in congenital heart disease.

  View details for DOI 10.1016/j.athoracsur.2012.05.096

  View details for Web of Science ID 000310439700031

  View details for PubMedID 22858270

• Late Outcomes in Patients Undergoing Aortopulmonary Window for Pulmonary Atresia/Stenosis and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Perry, S. B., Peng, L., Hanley, F. L. 2012; 94 (3): 842-849

  Abstract

  Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.

  View details for DOI 10.1016/j.athoracsur.2012.03.061

  View details for PubMedID 22857982

• 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

• Hypoplastic Left Heart Syndrome Current Considerations and Expectations JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Feinstein, J. A., Benson, D. W., Dubin, A. M., Cohen, M. S., Maxey, D. M., Mahle, W. T., Pahl, E., Villafane, J., Bhatt, A. B., Peng, L. F., Johnson, B. A., Marsden, A. L., Daniels, C. J., Rudd, N. A., Caldarone, C. A., Mussatto, K. A., Morales, D. L., Ivy, D. D., Gaynor, J. W., Tweddell, J. S., Deal, B. J., Furck, A. K., Rosenthal, G. L., Ohye, R. G., Ghanayem, N. S., Cheatham, J. P., Tworetzky, W., Martin, G. R. 2012; 59 (1): S1-S42

  Abstract

  In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

  View details for DOI 10.1016/j.jacc.2011.09.022

  View details for PubMedID 22192720

• 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

• Effect of pulmonary artery angioplasty on exercise function after repair of tetralogy of Fallot. American heart journal Sutton, N. J., Peng, L., Lock, J. E., Lang, P., Marx, G. R., Curran, T. J., O'Neill, J. A., Picard, S. T., Rhodes, J. 2008; 155 (1): 182-6

  Abstract

  The slope of the minute ventilation versus CO2 production relationship (VE/VCO2 slope) is an index of gas exchange efficiency during exercise. In patients with repaired tetralogy of Fallot (rTOF), it correlates negatively with exercise capacity and is one of the best predictors of peak oxygen consumption (VO2). In these patients, the magnitude of the VE/VCO2 slope is related to the severity of pulmonary blood flow maldistribution (PBFM). The purpose of this study was to determine whether, in patients with rTOF, improvements in PBFM after a successful balloon angioplasty procedure (BAP) result in improvements in peak VO2 and gas exchange during exercise.Seventeen patients with rTOF and residual pulmonary artery stenoses referred for BAP were recruited. Exercise tests were performed and PBFM determined before and after BAP.Nine patients (group 1) had a successful BAP (ie, improvement of >5 percentage points in PBFM); 8 did not (group 2). Patients in group 1 had significantly greater improvements in VE/VCO2 slope, peak VO2, and peak oxygen pulse (an index of forward stroke volume at peak exercise) than did patients in group 2. A significant correlation existed between the improvement in PBFM and the decline in the VE/VCO2 slope (r = -0.70, P = .002). Changes in peak oxygen pulse accounted for 89% of the improvement in peak VO2.In these patients, a successful BAP resulted in improved peak VO2 and more efficient gas exchange during exercise. The improvement in peak VO2 appeared to be mediated by an increase in forward stroke volume.

  View details for DOI 10.1016/j.ahj.2007.08.019

  View details for PubMedID 18082511

• Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a 15-year experience. Circulation Peng, L. F., McElhinney, D. B., Nugent, A. W., Powell, A. J., Marshall, A. C., Bacha, E. A., Lock, J. E. 2006; 113 (22): 2598-605

  Abstract

  The optimal treatment for dysfunctional right ventricle-to-pulmonary artery (RV-PA) conduits is unknown. Limited follow-up data on stenting of RV-PA conduits have been reported.Between 1990 and 2004, deployment of balloon-expandable bare stents was attempted in 242 obstructed RV-PA conduits in 221 patients (median age, 6.7 years). Acute hemodynamic changes after stenting included significantly decreased RV systolic pressure (89+/-18 to 65+/-20 mm Hg, P<0.001) and peak RV-PA gradient (59+/-19 to 27+/-14 mm Hg, P<0.001). There were no deaths, and, aside from 5 malpositioned stents requiring surgical removal, there were no serious procedural complications. During follow-up of 4.0+/-3.2 years, 9 patients died and 2 underwent heart transplantation, none related to catheterization or stent malfunction. During 155 follow-up catheterizations in 126 patients, the stent was redilated in 83 patients and additional stents were placed in 41. Stent fractures were diagnosed in 56 patients (43%) and associated with stent compression and substernal location but did not cause acute hemodynamic consequences. By Kaplan-Meier analysis, median freedom from conduit surgery after stenting was 2.7 years (3.9 years in patients >5 years), with younger age, homograft conduit, conduit diameter < or =10 mm, diagnosis other than tetralogy of Fallot, Genesis stent, higher prestent RV:aortic pressure ratio, and stent malposition associated with shorter freedom from surgery. Tricuspid regurgitation and RV function did not change between stent implantation and subsequent surgery.Conduit stenting is an effective interim treatment for RV-PA conduit obstruction and prolongs conduit lifespan in most patients. Stent fractures were common but not associated with significant complications or earlier conduit reoperation.

  View details for DOI 10.1161/CIRCULATIONAHA.105.607127

  View details for PubMedID 16735676

• Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Peng, Mcelhinney
• Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Circulation Peng, L.
• Endovascular stenting of obstructed right ventricle-to-pulmonary artery conduits: a fifteen-year experience Circulation Peng