Clinical Focus

  • Pediatric Cardiology

Academic Appointments

Professional Education

  • Fellowship: Baylor College of Medicine (2011) TX
  • Medical Education: Pritzker School of Medicine University of Chicago Registrar (2005) IL
  • Board Certification: American Board of Pediatrics, Pediatrics (2009)
  • Board Certification: American Board of Pediatrics, Pediatric Cardiology (2012)
  • Fellowship: Texas Children's Hospital Baylor College of Medicine (2012) TX
  • Residency: Stanford University Medical Center (2008) CA United States of America

All Publications

  • Comparative Costs of Management Strategies for Neonates With Symptomatic TetralogyofFallot. Journal of the American College of Cardiology O'Byrne, M. L., Glatz, A. C., Huang, Y. V., Kelleman, M. S., Petit, C. J., Qureshi, A. M., Shahanavaz, S., Nicholson, G. T., Batlivala, S., Meadows, J. J., Zampi, J. D., Law, M. A., Romano, J. C., Mascio, C. E., Chai, P. J., Maskatia, S., Asztalos, I. B., Beshish, A., Pettus, J., Pajk, A. L., Healan, S. J., Eilers, L. F., Merritt, T., McCracken, C. E., Goldstein, B. H. 2022; 79 (12): 1170-1180


    BACKGROUND: Recent data have demonstrated that overall mortality and adverse events are not significantly different for primary repair (PR) and staged repair (SR) approaches to management of neonates with symptomatic tetralogy of Fallot (sTOF). Cost data can be used to compare the relative value (cost for similar outcomes) of these approaches and are a potentially more sensitive measure of morbidity.OBJECTIVES: This study sought to compare the economic costs associated with PR and SR in neonates with sTOF.METHODS: Data from a multicenter retrospective cohort study of neonates with sTOF were merged with administrative data to compare total costs and cost per day alive over the first 18months of life in a propensity score-adjusted analysis. A secondary analysis evaluated differences in department-level costs.RESULTS: In total, 324 subjects from 6 centers from January 2011 to November 2017 were studied (40% PR). The 18-month cumulative mortality (P = 0.18), procedural complications (P = 0.10), hospital complications (P = 0.94), and reinterventions (P = 0.22) did not differ between PR and SR. Total 18-month costs for PR (median $179,494 [IQR: $121,760-$310,721]) were less than for SR (median: $222,799 [IQR: $167,581-$327,113]) (P< 0.001). Cost per day alive (P = 0.005) and department-level costs were also all lower for PR. In propensity score-adjusted analyses, PR was associated with lower total cost (cost ratio: 0.73; P< 0.001) and lower department-level costs.CONCLUSIONS: In this multicenter study of neonates with sTOF, PR was associated with lower costs. Given similar overall mortality between treatment strategies, this finding suggests that PR provides superior value.

    View details for DOI 10.1016/j.jacc.2021.12.036

    View details for PubMedID 35331412

  • The Impact of Maternal Hyperoxygenation on Myocardial Deformation and Loading Conditions in Fetuses With and Without Left-Heart Hypoplasia. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Cox, K. L., Morris, S. A., Tacy, T., Long, J., Becker, J., Schoppe Rdcs, L., Zhang Rdcs, J., Maskatia, S. A. 2022


    BACKGROUND: Maternal hyperoxygenation (MHO) is used in a variety of clinical applications, but its impact on fetal cardiovascular physiology is poorly understood. Our aims were: to describe the effects of MHO on myocardial deformation parameters and on ultrasound-based metrics of preload and afterload, and to assess the differential effect of MHO on fetuses with left heart hypoplasia (LHH). We hypothesized that the effects of MHO would be modulated by loading conditions, and that fetuses with LHH would be more sensitive to changes in preload and afterload induced by MHO.METHODS: We performed a post-hoc analysis of 36 fetal echocardiograms performed as part of a pilot study of MHO in LHH (n=9) and control (n=9) fetuses. Oxygen was administered via 8L face mask for 10 minutes. RV and LV longitudinal strain and strain rate, estimated aortic and pulmonary cardiac output, pulmonary vein velocity time integral (VTI) and pulsatility indices (PI) of the middle cerebral artery (MCA), pulmonary arteries (PA) and umbilical artery (UA) were measured at 3 time points: baseline, during MHO, and 10 minutes after removal of MHO.RESULTS: MHO induced decreases in LV strain and strain rate and increases in RV strain and strain rate. PA PI decreased and pulmonary vein VTI increased suggesting decreased pulmonary vascular resistance and increased pulmonary venous return. Most findings did not return to baseline after removal of MHO. We found no significant effect of MHO on MCA or UA PI. LHH cases demonstrated similar effects of MHO to control cases, with larger changes in pulmonary vein VTI and LV strain rate.CONCLUSION: The effects of MHO on LV and RV mechanics suggest that changes in deformation indices may be explained by increases in LV preload and decreases in RV afterload. The time period for recovery of fetal hemodynamics from MHO is ill-defined.

    View details for DOI 10.1016/j.echo.2022.03.015

    View details for PubMedID 35346806

  • Vivian Thomas, Eileen Saxon and neonatal cardiology. Seminars in perinatology Maskatia, S. A. 2022: 151589

    View details for DOI 10.1016/j.semperi.2022.151589

    View details for PubMedID 35393128

  • Coarctation of the aorta: Prenatal assessment, postnatal management and neonatal outcomes. Seminars in perinatology Arya, B., Maskatia, S. A. 2022: 151584


    Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates with severe Coa may be completely asymptomatic at birth, as the ductus arteriosus can provide flow to the lower body. Those who are not diagnosed prenatally may be diagnosed only after constriction of the ductus arteriosus, when they present in cardiogenic shock. This group has a higher risk for mortality and morbidity relative to those diagnosed prenatally. Despite the increasing practice of universal pulse oximetry screening, many cases with significant coarctation of the aorta still go undiagnosed in the newborn period. In this article, we present the pathophysiology, diagnosis, presentation, treatment and outcomes of Coa.

    View details for DOI 10.1016/j.semperi.2022.151584

    View details for PubMedID 35422354

  • mRNA Coronavirus-19 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study. The Journal of pediatrics Kohli, U., Desai, L., Chowdhury, D., Harahsheh, A. S., Yonts, A. B., Ansong, A., Sabati, A., Nguyen, H. H., Hussain, T., Khan, D., Parra, D. A., Su, J. A., Patel, J. K., Ronai, C., Bohun, M., Freij, B. J., O'Connor, M. J., Rosanno, J. W., Gupta, A., Salavitabar, A., Dorfman, A. L., Hansen, J., Frosch, O., Profita, E. L., Maskatia, S., Thacker, D., Shrivastava, S., Harris, T., Feingold, B., Berger, S., Campbell, M., Idriss, S. F., Das, S., Renno, M. S., Knecht, K., Asaki, S. Y., Patel, S., Ashwath, R., Shih, R., Phillips, J., Das, B., Ramachandran, P., Sagiv, E., Bhat, A. H., Johnson, J. N., Taggart, N., Imundo, J., Nakra, N., Behere, S., Patel, A., Agarwal, A., Aljemmali, S., Lang, S., Batlivala, S., Forsha, D. E., Conners, G. P., Shaw, J., Smith, F. C., Pauliks, L., Vettukattil, J., Shaffer, K., Cheang, S., Voleti, S., Shenoy, R., Komarlu, R., Ryan, S. J., Snyder, C., Bansal, N., Sharma, M., Robinson, J., Arnold, S. R., Salvatore, C. M., Kumar, M., Fremed, M., Glickstein, J. S., Perrotta, M., Orr, W., Rozema, T., Thirumoorthi, M., Mullett, C. J., Ang, J. Y. 1800


    In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA COVID-19 vaccine-associated myopericarditis (VAM) was noted. Only one adolescent with life-threatening complications was reported with no deaths at any of the participating institutions.

    View details for DOI 10.1016/j.jpeds.2021.12.025

    View details for PubMedID 34952008

  • Left Subclavian Artery Isolation with Right Aortic Arch and D-Transposition of the Great Arteries. CASE (Philadelphia, Pa.) Hansen, K., Dhillon, G., Ma, M., Maskatia, S. A., Su, L., Vasanawala, S., Punn, R. 1800; 5 (6): 392-398

    View details for DOI 10.1016/

    View details for PubMedID 34993370

  • 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


    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

  • COVID-19 Vaccination-Associated Myocarditis in Adolescents. Pediatrics Jain, S. S., Steele, J. M., Fonseca, B., Huang, S., Shah, S., Maskatia, S. A., Buddhe, S., Misra, N., Ramachandran, P., Gaur, L., Eshtehardi, P., Anwar, S., Kaushik, N., Han, F., Chaudhuri, N. R., Grosse-Wortmann, L. 2021

    View details for DOI 10.1542/peds.2021-053427

    View details for PubMedID 34389692

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


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

    View details for DOI 10.1002/jmri.27729

    View details for PubMedID 34037289

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


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

    View details for DOI 10.1053/j.semtcvs.2021.03.044

    View details for PubMedID 33971298

  • The Impact of Maternal Preeclampsia and Hyperglycemia on the Cardiovascular Health of the Offspring: A Systematic Review and Meta-Analysis. American journal of perinatology Hoodbhoy, Z., Mohammed, N., Nathani, K. R., Sattar, S., Chowdhury, D., Maskatia, S., Tierney, S., Hasan, B., Das, J. K. 2021


    OBJECTIVES: The objective of this review was to assess the impact of maternal preeclampsia or hyperglycemia on the body composition and cardiovascular health in the offspring.STUDY DESIGN: We conducted a systematic review utilizing PubMed, EBSCO, CINAHLPlus, Cochrane Library, and Web of Science to include all studies assessing the impact of preeclampsia/eclampsia and/or gestational/pregestational diabetes mellitus on the health of the offspring (children <10 years of age). The health measures included anthropometry, cardiac dimensions and function, and vascular function. We performed a meta-analysis using Review Manager software and computed net risk ratio (RR) with 95% confidence interval (CI) for dichotomous data and mean difference (MD) with 95% CI for continuous data.RESULTS: There were 6,376 studies in total, of which 45 were included in the review and 40 in the meta-analysis. The results demonstrated higher birth weight (MD: 0.12kg; 95% CI: 0.06-0.18) and systolic and diastolic blood pressure (BP; MD: 5.98mmHg; 95% CI: 5.64-6.32 and MD: 3.27mmHg; 95% CI: 0.65-5.89, respectively) in the offspring of mothers with gestational diabetes compared to controls. In contrast, the offspring of mothers with preeclampsia had lower birth weight (MD: -0.41kg; 95% CI: -0.7 to -0.11); however, they had increased systolic (MD: 2.2mmHg; 95% CI: 1.28-3.12) and diastolic BP (MD: 1.41mmHg; 95% CI: 0.3-2.52) compared to controls. There is lack of data to conduct a meta-analysis of cardiac morphology, functional, and vascular imaging parameters.CONCLUSION: These findings suggest that the in-utero milieu can have a permanent impact on the body composition and vascular health of the offspring. Future work warrants multicenter prospective studies to understand the mechanism and the actual effect of exposure to maternal hyperglycemia and high BP on the cardiovascular health of the offspring and long-term outcomes.KEY POINTS: · Adverse in-utero exposures may have an impact on cardiovascular risk in children.. · Maternal hyperglycemia/preeclampsia lead to changes in birthweight and BP.. · Limited echocardiographic and vascular imaging data in these cohorts necessitates future work..

    View details for DOI 10.1055/s-0041-1728823

    View details for PubMedID 33940650

  • Postpartum depression in mothers with pregnancies complicated by fetal cardiac anomaly. Journal of perinatology : official journal of the California Perinatal Association Waldrop, A. R., Sherwin, E. B., Anderson, J. N., Boissiere, J. C., Hintz, S. R., Maskatia, S. A., Girsen, A. I., Blumenfeld, Y. J. 2021


    OBJECTIVE: To investigate factors associated with positive postpartum depression (PPD) screen in pregnancies complicated by fetal congenital cardiac anomaly.STUDY DESIGN: We reviewed all records of pregnancies complicated by fetal congenital cardiac anomaly receiving prenatal, intrapartum and postpartum care at our single center, October 2016-October 2019. Maternal, obstetric, and neonatal data were compared between women with and without a positive PPD screen at the 6-week postpartum visit.RESULTS: Out of 415 women referred for fetal congenital cardiac anomaly, 86 women had complete inclusion criteria. Twenty-four women (28%) had a positive PPD screen. The frequencies of planned future infant surgery (73.9 vs. 26.2%, p=0.01) and neonatal death prior to postpartum visit (12.5 vs. 0%, p=0.02) were significantly higher among women with a positive PPD screen.CONCLUSION: In pregnancies complicated by fetal congenital cardiac anomaly, mothers of infants with planned future surgery or neonatal death are at significant risk for postpartum depression.

    View details for DOI 10.1038/s41372-021-01017-6

    View details for PubMedID 33712713

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


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

    View details for DOI 10.1016/j.jpeds.2021.06.047

    View details for PubMedID 34181988

  • Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot. Journal of the American College of Cardiology Goldstein, B. H., Petit, C. J., Qureshi, A. M., McCracken, C. E., Kelleman, M. S., Nicholson, G. T., Law, M. A., Meadows, J. J., Zampi, J. D., Shahanavaz, S. n., Mascio, C. E., Chai, P. J., Romano, J. C., Batlivala, S. P., Maskatia, S. A., Asztalos, I. B., Kamsheh, A. M., Healan, S. J., Smith, J. D., Ligon, R. A., Pettus, J. A., Juma, S. n., Raulston, J. E., Hock, K. M., Pajk, A. L., Eilers, L. F., Khan, H. Q., Merritt, T. C., Canter, M. n., Juergensen, S. n., Rinderknecht, F. A., Bauser-Heaton, H. n., Glatz, A. C. 2021; 77 (8): 1093–1106


    Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

    View details for DOI 10.1016/j.jacc.2020.12.048

    View details for PubMedID 33632484

  • Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease. Pediatric cardiology Swanson, T. M., Patel, A., Baxter, A. J., Morris, S. A., Maskatia, S. A., Lantos, J. D. 2020


    In order toevaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions,we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion. We surveyed 126 cardiologists and 9 cardiac surgeons at four tertiary referral centers. Overall response rate was 77%. Greater than 70% of physicians would seek legal action and mandate surgeryfor the following lesions: ventricular septal defect, coarctation of the aorta, complete atrioventricular canal, transposition of the great arteries, tetralogy of Fallot, and unobstructed total anomalous pulmonary venous return. Surgery for all of these lesions has reported mortality rates of<5%. Physicians were less likely to seek legal action when parents refused surgery for Shone complex, any single ventricle lesion, or any congenital heart disease accompanied by Trisomy 13 or Trisomy18. Among experts in pediatric cardiology, there is widespread agreement about the appropriate response to parental refusal of surgery for most congenital heart lesions, and these lesions tended to be heart defects with lower surgical mortality rates. Lesions for which there was greater consensus among experts were those with the best outcomes. There was less consensus for lesions with higher mortality rates. Such surveys, revealing disagreement among expert professionals, can provide an operational definition of the current professional "gray zone" in which parental preferences should determine treatment.

    View details for DOI 10.1007/s00246-020-02367-2

    View details for PubMedID 32419096

  • Direct measurement of atrioventricular valve regurgitant jets using 4D flow cardiovascular magnetic resonance is accurate and reliable for children with congenital heart disease: a retrospective cohort study. Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance Jacobs, K., Rigdon, J., Chan, F., Cheng, J. Y., Alley, M. T., Vasanawala, S., Maskatia, S. A. 2020; 22 (1): 33


    BACKGROUND: 3D-time resolved flow (4DF) cardiovascular magnetic resonance (CMR) with retrospective analysis of atrioventricular valve regurgitation (AVVR) allows for internal validation by multiple direct and indirect methods. Limited data exist on direct measurement of AVVR by 4DF CMR in pediatric congenital heart disease (CHD). We aimed to validate direct measurement of the AVVR jet as accurate and reliable compared to the volumetric method (clinical standard by 2D CMR) and as a superior method of internal validation than the annular inflow method.METHODS: We identified 44 consecutive patients with diverse CHD referred for evaluation of AVVR by CMR. 1.5T or 3T scanners, intravenous contrast, and a combination of parallel imaging and compressed sensing were used. Four methods of measuring AVVR volume (RVol) were used: volumetric method (VOL; the clinical standard)=stroke volume by 2D balanced steady-state free precession - semilunar valve forward flow (SLFF); annular inflow method (AIM)=atrioventricular valve forward flow [AVFF] - semilunar valve net flow (SLNF); and direct measurement (JET). AVFF was measured using static and retrospective valve tracking planes. SLFF, SLNF, AVFF, and JET were measured by 4DF phase contrast. Regurgitant fraction was calculated as [RVol/(RVol+SLNF)]*100. Statistical methods included Spearman, Wilcoxon rank sum test/Student paired t-test, Bland Altman analysis, and intra-class coefficient (ICC), where appropriate.RESULTS: Regurgitant fraction by JET strongly correlated with the indirect methods (VOL and AIM) (rho=0.73-0.80, p<0.001) and was similar to VOL with a median difference (interquartile range) of -1.5% (-8.3-7.2%; p=0.624). VOL had weaker correlations with AIM and JET (rho=0.69-0.73, p<0.001). AIM underestimated RF by 3.6-6.9% compared to VOL and JET, p<0.03. Intra- and inter- observer reliability were excellent for all methods (ICC 0.94-0.99). The mean (±standard deviation) inter-observer difference for VOL was 2.4% (±5.1%), p<0.05.CONCLUSIONS: In a diverse cohort of pediatric CHD, measurement of AVVR using JET is accurate and reliable to VOL and is a superior method of internal validation compared to AIM. This study supports use of 4DF CMR for measurement of AVVR, obviating need for expert prospective prescription during image acquisition by 2D CMR.

    View details for DOI 10.1186/s12968-020-00612-4

    View details for PubMedID 32404159

  • COVID-19 and Kawasaki Disease: Novel Virus and Novel Case. Hospital pediatrics Jones, V. G., Mills, M. n., Suarez, D. n., Hogan, C. A., Yeh, D. n., Bradley Segal, J. n., Nguyen, E. L., Barsh, G. R., Maskatia, S. n., Mathew, R. n. 2020

    View details for DOI 10.1542/hpeds.2020-0123

    View details for PubMedID 32265235

  • 4D flow vs. 2D cardiac MRI for the evaluation of pulmonary regurgitation and ventricular volume in repaired tetralogy of Fallot: a retrospective case control study. The international journal of cardiovascular imaging Jacobs, K. G., Chan, F. P., Cheng, J. Y., Vasanawala, S. S., Maskatia, S. A. 2020


    Lengthy exams and breath-holding limit the use of pediatric cardiac MRI (CMR). 3D time-resolved flow MRI (4DF) is a free-breathing, single-sequence exam that obtains magnitude (anatomic) and phase contrast (PC) data. We compare the accuracy of gadobenate dimeglumine-enhanced 4DF on a 1.5T magnet to 2D CMR in children with repaired tetralogy of Fallot (rTOF) to measure pulmonary net flow (PNF) as a reflection of pulmonary regurgitation, forward flow (FF) and ventricular volumetry. Thirty-four consecutive cases were included. 2D PCs were obtained at the valve level. Using 4DF, we measured PNF at the valve and at the main and branch pulmonary arteries. PNF measured at the valve by 4DF demonstrated the strongest correlation (r=0.87, p<0.001) and lowest mean difference (3.5±9.4mL/beat) to aortic net flow (ANF). Semilunar FF and stroke volume of the respective ventricle demonstrated moderate-strong correlation by 4DF (r=0.66-0.81, p<0.001) and strong correlation by 2D (r=0.81-0.84, p<0.001) with similar correlations and mean differences between techniques (p>0.05). Ventricular volumes correlated strongly between 2D and 4DF (r=0.75-0.96, p<0.001), though 4DF overestimated right ventricle volumes by 11.8-19.2mL/beat. Inter-rater reliability was excellent for 2D and 4DF volumetry (ICC=0.91-0.99). Ejection fraction moderately correlated (r=0.60-0.75, p<0.001) with better reliability by 4DF (ICC: 0.80-0.85) than 2D (ICC: 0.69-0.89). 4DF exams were shorter than 2D (9 vs. 71min, p<0.001). 4DF provides highly reproducible and accurate measurements of flow with slight overestimation of RV volumes compared to 2D in pediatric rTOF. 4DF offers important advantages in this population with long-term monitoring needs.

    View details for DOI 10.1007/s10554-019-01751-1

    View details for PubMedID 31894524

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


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

    View details for DOI 10.1053/j.semtcvs.2020.11.009

    View details for PubMedID 33181302

  • Postoperative Recovery of Left Ventricular Function following Repair of Large Ventricular Septal Defects in Infants. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Adamson, G. T., Arunamata, A., Tacy, T. A., Silverman, N. H., Ma, M., Maskatia, S. A., Punn, R. 2019


    INTRODUCTION: Early postoperative left ventricular (LV) dysfunction is observed following repair of large ventricular septal defects (VSDs), but the frequency and rate of recovery of LV function are unknown. This study aims to characterize the incidence and rate of recovery of postoperative LV dysfunction following repair of large VSDs and to improve understanding of LV mechanics before and after VSD repair.METHODS: Infants who underwent surgical repair of an isolated large VSD were included. Pre- and postoperative echocardiographic images were reviewed, and LV function was assessed by both conventional echocardiography and speckle-tracking strain analyses. Postoperative LV dysfunction was defined as an LV ejection fraction (LVEF)<50% using the 5/6 area-length method. Echocardiograms were reviewed to assess LVEF and LV volume through 1-year follow-up.RESULTS: Of 104 infants evaluated (median age, 0.31 [0.22, 0.56] years), all had normal preoperative LVEF and 39 (38%) had postoperative LV dysfunction. Follow-up echocardiograms were available in 31 (80%) patients, all of whom had LVEF>50% within 9months of surgery. Lower preoperative apical four-chamber longitudinal strain (A4LS) and greater LV end-diastolic volume indexed to body surface area1.38 were independently associated with postoperative LV dysfunction. An absolute preoperative A4LS < 16.7% (area under the curve=0.87; 95% CI, 0.78-0.95; P<.001) was most strongly associated with postoperative dysfunction.CONCLUSIONS: Infants with LV dysfunction following repair of large VSDs recover function within 9months. Preoperative A4LS can be helpful to detect subclinical LV dysfunction in the setting of a large hemodynamically significant VSD and guide postoperative expectations for providers and families.

    View details for DOI 10.1016/j.echo.2019.10.003

    View details for PubMedID 31866322

  • In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome. Prenatal diagnosis Mardy, C., Blumenfeld, Y. J., Arunamata, A. A., Girsen, A. I., Sylvester, K. G., Halabi, S., Rubesova, E., Hintz, S. R., Tacy, T. A., Maskatia, S. A. 2019


    INTRODUCTION: The clinical importance of mass effect from congenital lung masses on the fetal heart is unknown. We aimed to report cardiac measurements in fetuses with congenital lung masses, and correlate lung mass severity/size with cardiac dimensions and clinical outcomes.METHODS: Cases were identified from our institutional database between 2009 and 2016. We recorded: atrioventricular valve (AVVz) annulus dimensions and ventricular widths (VWz) converted into z-scores, ratio of aortic to total cardiac output (AoCO), lesion side, and congenital pulmonary airway malformation volume ratio (CVR). Respiratory intervention (RI) was defined as: intubation, ECMO use or surgical intervention prior to discharge.RESULTS: Fifty-two fetuses comprised the study cohort. Mean AVVz and VWz were below expected for gestational age. CVR correlated with ipsilateral AVVz (RS =-0.59, p<0.001) and ipsilateral VWz (-0.59, p<0.001). Lower AVVz, AoCO, and higher CVR were associated with RI. No patient had significant structural heart disease identified postnatally.CONCLUSION: In fetuses with left-sided lung masses, ipsilateral cardiac structures tend to be smaller, but in our cohort there were no patients with structural heart disease. However, smaller left-sided structures may contribute to the need for RI that affects a portion of these fetuses.

    View details for DOI 10.1002/pd.5612

    View details for PubMedID 31742724

  • Infundibular sparing versus transinfundibular approach to the repair of tetralogy of Fallot. Congenital heart disease Olive, M. K., Fraser, C. D., Kutty, S., McKenzie, E. D., Hammel, J. M., Krishnamurthy, R., Dodd, N. A., Maskatia, S. A. 2019; 14 (6): 1149–56


    INTRODUCTION: The right ventricular infundibular sparing approach (RVIS) to the repair of tetralogy of Fallot (TOF) avoids a full-thickness ventricular incision, typically utilized in the transinfundibular (TI) method.METHODS: We performed a retrospective, age-matched cohort study of patients who underwent RVIS at Texas Children's Hospital or TI at Children's Hospital Medical Center in Nebraska and subsequently underwent cardiac magnetic resonance imaging (CMR). We compared right ventricular end-diastolic and systolic volumes indexed to body surface area (RVEDVi and RVESVi) and right ventricular ejection fraction (RVEF) as primary endpoints. Secondary endpoints were indexed left ventricular diastolic and systolic volume (LVEDVi and LVESVi), left ventricular ejection fraction (LVEF), right ventricular (RV) sinus ejection fraction (EF) and RV outflow tract EF (RVOT EF).RESULTS: Seventy-nine patients were included in the analysis; 40 underwent RVIS and 39 underwent TI repair. None of the patients in the TI repair group had an initial palliation with a systemic to pulmonary arterial shunt compared to seven (18%) in the RVIS group (P<.01). There was no appreciable difference in RVEDVi (122±29 cc/m2 vs 130±29cc/m2 , P=.59) or pulmonary regurgitant fraction (40±13 vs 37±18, P=.29) between the RVIS and TI groups. Compared to the TI group, the RVIS group had higher RVEF (54±6% vs 44±9%, P<.01), lower RVESV (57±17 cc/m2 vs 67±25 cc/m2 , P=.03), higher LVEF (61±11% vs 54±8%, P<.01), higher RVOT EF (47±12% vs 41±11%, P=.03), and higher RV sinus EF (56±5% vs 49±6%, P<.01) CONCLUSIONS: In this selected cohort, patients who underwent RVIS repair for TOF had higher right and left ventricular ejection fraction compared to those who underwent TI repair.

    View details for DOI 10.1111/chd.12863

    View details for PubMedID 31917528

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


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

    View details for DOI 10.1016/j.jtcvs.2019.06.110

    View details for PubMedID 31585750

  • Fetal Echocardiographic Parameters and Surgical Outcomes in Congenital Left-Sided Cardiac Lesions. Pediatric cardiology Edwards, L. A., Arunamata, A. n., Maskatia, S. A., Quirin, A. n., Bhombal, S. n., Maeda, K. n., Tacy, T. A., Punn, R. n. 2019


    This study aimed to evaluate fetal echocardiographic parameters associated with neonatal intervention and single-ventricle palliation (SVP) in fetuses with suspected left-sided cardiac lesions. Initial fetal echocardiograms (1/2002-1/2017) were interpreted by the contemporary fetal cardiologist as coarctation of the aorta (COA), left heart hypoplasia (LHH), hypoplastic left heart syndrome (HLHS), mitral valve hypoplasia (MVH) ± stenosis, and aortic valve hypoplasia ± stenosis (AS). The cohort comprised 68 fetuses with suspected left-sided cardiac lesions (COA n = 15, LHH n = 9, HLHS n = 39, MVH n = 1, and AS n = 4). Smaller left ventricular (LV) length Z score, aortic valve Z score, ascending aorta Z score, and aorta/pulmonary artery ratio; left-to-right shunting at the foramen ovale; and retrograde flow in the aortic arch were associated with the need for neonatal intervention (p = 0.005-0.04). Smaller mitral valve (MV) Z score, LV length Z score, aortic valve Z score, ascending aorta Z score, aorta/pulmonary artery ratio, and LV ejection fraction, as well as higher tricuspid valve-to-MV (TV/MV) ratio, right ventricular-to-LV (RV/LV) length ratio, left-to-right shunting at the foramen ovale, abnormal pulmonary vein Doppler, absence of prograde aortic flow, and retrograde flow in the aortic arch were associated with SVP (p < 0.001-0.008). The strongest independent variable associated with SVP was RV/LV length ratio (stepwise logistical regression, p = 0.03); an RV/LV length ratio > 1.28 was associated with SVP with a sensitivity of 76% and specificity of 96% (AUC 0.90, p < 0.001). A fetal RV/LV length ratio of > 1.28 may be a useful threshold for identifying fetuses requiring SVP.

    View details for DOI 10.1007/s00246-019-02155-7

    View details for PubMedID 31338561

  • Human Milk Use in the Preoperative Period Is Associated with a Lower Risk for Necrotizing Enterocolitis in Neonates with Complex Congenital Heart Disease. The Journal of pediatrics Cognata, A. n., Kataria-Hale, J. n., Griffiths, P. n., Maskatia, S. n., Rios, D. n., O'Donnell, A. n., Roddy, D. J., Mehollin-Ray, A. n., Hagan, J. n., Placencia, J. n., Hair, A. B. 2019


    To evaluate the hypothesis that feeding volumes exceeding 100 mL/kg/d and exposure to cow's milk formula preoperatively increase the risk for preoperative necrotizing enterocolitis (NEC) in infants with complex congenital heart disease.All infants, of any gestational age, with an isolated cardiac lesion at high risk for NEC (ductal-dependent lesions, transposition of the great arteries, truncus arteriosus, and aorto-pulmonary window) admitted to Texas Children's Hospital from 2010 to 2016 were included. NEC was defined based on the modified Bell criteria. Feeding regimen information and relevant covariates were collected. Logistic regression was used to evaluate the association of feeding regimen and other potential risk factors with NEC.In this single-center, retrospective cohort of 546 infants, 3.3% developed Bell stage I-III NEC preoperatively. An exclusive unfortified human milk diet was associated with a significantly lower risk of preoperative NEC (OR 0.17, 95% CI 0.04-0.84, P = .03) in a multivariable regression model controlling for cardiac lesion, race, feeding volume, birth weight small for gestational age, inotrope use presurgery/pre-NEC, and prematurity. Feeding volumes exceeding 100 mL/kg/d were associated with a significantly greater risk of preoperative NEC (OR 3.05, 95% CI 1.19-7.90, P = .02).The findings suggest that an unfortified exclusive human milk diet may reduce the risk of preoperative NEC in infants with complex congenital heart disease.

    View details for DOI 10.1016/j.jpeds.2019.08.009

    View details for PubMedID 31561958

  • Weakly supervised classification of rare aortic valve malformations using unlabeled cardiac MRI sequences Nature Communications Fries, J. A., Varma, P., Chen, V. S., Xiao, K., Tejeda, H., Saha, P., Dunnmon, J., Chubb, H., Maskatia, S., Fiterau, M., Delp, S., Ashley, E., Ré, C., Priest, J. R. 2019; 10
  • The impact of fetal endoscopic tracheal occlusion in isolated left-sided congenital diaphragmatic hernia on left-sided cardiac dimensions PRENATAL DIAGNOSIS Dhillon, G. S., Maskatia, S. A., Loar, R. W., Colquitt, J. L., Mehollin-Ray, A. R., Ruano, R., Belfort, M. A., Olutoye, O. O., Kailin, J. A. 2018; 38 (11): 812–20


    Fetal endoscopic tracheal occlusion (FETO) is offered to fetuses with congenital diaphragmatic hernia (CDH) and severe lung hypoplasia to promote lung growth and may secondarily affect left heart growth. The effects of FETO on left heart hypoplasia (LHH) are not described post-CDH repair.A retrospective analysis was performed for fetuses with left-sided CDH who underwent FETO and severity-matched controls from 2007 to 2016 at our institution. Echocardiographic, ultrasound, and MRI data were reviewed. Left heart dimensions were assessed prenatally and postnatally. Primary clinical outcome evaluated was death.Twelve FETO patients and 18 controls were identified. Fetal LHH was noted in both groups and worsened after FETO. Postnatal mitral valve dimensions were larger in the FETO group pre-CDH repair (P = .03). Post-CDH repair, mitral valve and left ventricular dimensions were not significantly different between groups (P = .79 and P = .63 respectively) while FETO aortic valve dimensions were smaller (P = .04). Extracorporeal membrane oxygenation use was lower in the FETO group. No associations were found between left heart dimensions and outcomes.Although increased lung growth was seen after FETO, fetal LHH persisted with relative normalization seen post-repair. Persistent LHH post-FETO could be secondary to a small contribution of pulmonary venous return to the fetal left heart and increased intrathoracic pressures post-FETO.

    View details for PubMedID 30047996

  • Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study. Congenital heart disease Maskatia, S. A., Petit, C. J., Travers, C. D., Goldberg, D. J., Rogers, L. S., Glatz, A. C., Qureshi, A. M., Goldstein, B. H., Ao, J., Sachdeva, R. 2018


    BACKGROUND: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.METHODS: We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1-year or prior to Glenn circulation).RESULTS: Eighty-one patients were included. At last follow-up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left-to-right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z-score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P < .001) were associated with larger increases in RV area. Baseline RV area ≥6 cm2 /m2 had 93% sensitivity and 80% specificity for identifying patients who ultimately achieved 2V circulation. All patients with RV area ≥8 cm2 /m2 at follow up achieved 2V circulation. This finding was confirmed in a validation cohort from a separate center (N = 25). Factors associated with achieving RV area ≥8 cm2 /m2 included larger TV z-score (P = .004), ≥ moderate baseline TR (P = .031), and ≥ moderate postdecompression pulmonary regurgitation (P = .002).CONCLUSIONS: Patients with PA/IVS and smaller TV annuli are at risk for poor RV growth. Volume-loading conditions signal increased capacity for growth sufficient for 2V circulation.

    View details for PubMedID 30238627

  • Cyanotic congenital heart disease following fertility treatments in the United States from 2011 to 2014 HEART Shamshirsaz, A. A., Bateni, Z. H., Sangi-Haghpeykar, H., Arian, S. E., Erfani, H., Shamshirsaz, A. A., Abuhamad, A., Fox, K. A., Ramin, S. M., Moaddab, A., Maskatia, S. A., Salmanian, B., Lopez, K. N., Hosseinzadeh, P., Schutt, A. K., Nassr, A. A., Espinoza, J., Dildy, G. A., Belfort, M. A., Clark, S. L. 2018; 104 (11): 945–48


    To examine the risk for cyanotic congenital heart diseases (CCHDs) among live births in the USA, resulting from various forms of infertility treatments.This study is a cross-sectional analysis of live births in the USA from 2011 to 2014. Infertility treatments are categorised into two of the following groups on birth certificates: assisted reproductive technology (ART) fertility treatment (surgical egg removal; eg, in vitro fertilisation and gamete intrafallopian transfer) and non-ART fertility treatment (eg, medical treatment and intrauterine insemination). We compared the risk for CCHD in ART and non-ART fertility treatment groups with those infants whose mothers received no documented fertility treatment and were naturally conceived (NC).Among 14 242 267 live births from 2011 to 2014, a total of 101 494 live births were in the ART and 81 242 resulted from non-ART fertility treatments. CCHD prevalence in ART, non-ART and NC groups were 393/100 892 (0.39%), 210/80 884 (0.26%) and 10 749/14 020 749 (0.08%), respectively. As compared with naturally conceiving infants, risk for CCHD was significantly higher among infants born in ART (adjusted relative risk (aRR) 2.4, 95% CI 2.1 to 2.7) and non-ART fertility treatment groups (aRR 1.9, 95% CI 1.6 to 2.2). Absolute risk increase in CCHD due to ART and non-ART treatments were 0.03% and 0.02%, respectively. A similar pattern was observed when the analysis was restricted to twins, newborns with birth weights under 1500 g and gestational age of less than 32 weeks.Our findings suggest an increased risk for CCHD in infants conceived after all types of infertility treatment.

    View details for PubMedID 29146625

  • The effect of maternal hyperoxygenation on fetal myocardial mechanics and hemodynamics in control fetuses and those with left-heart hypoplasia Cox, K., Morris, S. A., Becker, J., Tacy, T., Sakarovitch, C., Maskatia, S. A. MOSBY-ELSEVIER. 2018: S266
  • Chronic Maternal Hyperoxygenation and Effect on Cerebral and Placental Vasoregulation and Neurodevelopment in Fetuses with Left Heart Hypoplasia. Fetal diagnosis and therapy Edwards, L. A., Lara, D. A., Sanz Cortes, M. n., Hunter, J. V., Andreas, S. n., Nguyen, M. J., Schoppe, L. J., Zhang, J. n., Smith, E. M., Maskatia, S. A., Sexson-Tejtel, S. K., Lopez, K. N., Lawrence, E. J., Wang, Y. n., Challman, M. n., Ayres, N. A., Altman, C. A., Aagaard, K. n., Becker, J. A., Morris, S. A. 2018: 1–13


    In a pilot study of chronic maternal hyperoxygenation (CMH) in left heart hypoplasia (LHH), we sought to determine effect estimates of CMH on head size, vascular resistance indices, and neurodevelopment compared to controls.Nine gravidae meeting the inclusion criteria (fetal LHH, ≥25.9 weeks' gestation, and ≥10% increase in percent aortic flow after acute hyperoxygenation) were prospectively enrolled. Controls were 9 contemporary gravidae with fetal LHH without CMH. Brain growth and Doppler-derived estimates of fetal cerebrovascular and placental resistance were blindly evaluated and compared using longitudinal regression. Postnatal anthropomorphic and neurodevelopmental assessments were compared.There was no difference in baseline fetal measures between groups. There was significantly slower biparietal diameter (BPD) growth in the CMH group (z-score change -0.03 ± 0.02 vs. +0.09 ± 0.05 units/week, p = 0.02). At 6 months postnatal age, the mean head circumference z-score in the CMH group was smaller than that of controls (-0.20 ± 0.58 vs. +0.85 ± 1.11, p = 0.048). There were no differences in neurodevelopmental testing at 6 and 12 months.In this pilot study, relatively diminished fetal BPD growth and smaller infant head circumference z-scores at 6 months were noted with in utero CMH exposure.

    View details for PubMedID 30223262

  • The pediatric echocardiography Boot Camp: Four-year experience and impact on clinical performance ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Maskatia, S. A., Cabrera, A. G., Morris, S. A., Altman, C. A. 2017; 34 (10): 1486–94


    We previously reported on the short-term impact of an echocardiography "Boot Camp" on a single class of cardiology fellows (CF). The impact of the Boot Camp on performance throughout fellowship is unknown.We enrolled four classes of CFs and two classes of cardiac ICU fellows (CVs) prospectively into the Boot Camp and compared CFs to a historical cohort. Experience with echocardiography was surveyed. Outcome measures included written pre- and post-Camp exams, a performance based test (PBT), self-efficacy assessments, numbers of echocardiograms performed, and echocardiogram quality during the last 3 months of fellowship.A total of 25 CFs and 7 CVs participated in the Boot Camp from July 2012-July 2015. Median experience score was 13/40 (4-23). Median self-efficacy improved from 22/147 (range 21-45) to 90/147 (range 49-133) (P=<.001), and written scores from 14/29 (8-24) to 24/29 (13-29) (P<.001). CFs who completed the Boot Camp performed more independent echocardiograms compared to controls at the end of the 1st (37.7±12.2 vs 28.2±12.1, P=.15), 2nd (71.3±24.4 vs 47.6±16.0, P=.044), and third year of fellowship (130.4±44.0 vs 100.0±29.3, P=.230), and on average achieved 150 total echocardiograms in the 4.8th quarter compared to the 7.8th quarter in controls, P=.053. 2D quality scores were higher and shortening fraction more often obtained in echocardiograms performed by Boot Camp CFs compared to controls.The pediatric echocardiography Boot Camp improved self-efficacy, acquisition, and retention of echocardiography skills and knowledge, and increased echocardiogram performance. Observed differences between Boot Camp and control CFs appear to wane across fellowship.

    View details for PubMedID 28980410

  • Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes PRENATAL DIAGNOSIS Kailin, J. A., Dhillon, G. S., Maskatia, S. A., Cass, D. L., Shamshirsaz, A. A., Mehollin-Ray, A. R., Cassady, C. I., Ayres, N. A., Wang, Y., Belfort, M. A., Olutoye, O. O., Ruano, R. 2017; 37 (5): 502-509


    Fetuses with congenital diaphragmatic hernia (CDH) demonstrate varying degrees of left heart hypoplasia. Our study assesses the relationship between fetal left-sided cardiac structural dimensions, lung size, percentage liver herniation, lung-to-head ratio, postnatal left-sided cardiac structural dimensions, and postnatal outcomes.We performed a retrospective cohort study of fetuses with left-sided CDH who had prenatal echocardiographic, ultrasound, and magnetic resonance imaging examinations at our institution between January 2007 and March 2015. Postnatal outcomes assessed include use of inhaled nitric oxide (iNO), use of extracorporeal membrane oxygenation, and death.Fifty-two fetuses with isolated left-sided CDH were included. Multivariate logistic regression models indicated that smaller fetal aortic valve z-score was associated with postnatal use of iNO (p = 0.03). Fetal mitral valve z-score correlated with lung-to-head ratio (p = 0.04), postnatal mitral valve z-score correlated with percent liver herniation (p = 0.03), and postnatal left ventricular end-diastolic dimension z-score correlated with liver herniation <20% (p = 0.04).We identified associations between smaller fetal left-sided cardiac structural dimensions and classic CDH indices. Smaller aortic valve z-score was associated with iNO use; however, left heart dimensions showed no association with extracorporeal membrane oxygenation or mortality. Further study into the impact of left-sided hypoplasia on outcomes in CDH is worthy of evaluation in a larger, prospective study. © 2017 John Wiley & Sons, Ltd.

    View details for DOI 10.1002/pd.5045

    View details for Web of Science ID 000401561200012

    View details for PubMedID 28370263

  • Outcomes After Decompression of the Right Ventricle in Infants With Pulmonary Atresia With Intact Ventricular Septum Are Associated With Degree of Tricuspid Regurgitation: Results From the Congenital Catheterization Research Collaborative CIRCULATION-CARDIOVASCULAR INTERVENTIONS Petit, C. J., Glatz, A. C., Qureshi, A. M., Sachdeva, R., Maskatia, S. A., Justino, H., Goldberg, D. J., Mozumdar, N., Whiteside, W., Rogers, L. S., Nicholson, G. T., McCracken, C., Kelleman, M., Goldstein, B. H. 2017; 10 (5)


    Outcomes after right ventricle (RV) decompression in infants with pulmonary atresia with intact ventricular septum vary widely. Descriptions of outcomes are limited to small single-center studies.Neonates undergoing RV decompression for pulmonary atresia with intact ventricular septum were included from 4 pediatric centers. Primary end point was reintervention post-RV decompression; secondary end points included circulation type at latest follow-up. Ninety-nine patients (71 with pulmonary atresia with intact ventricular septum and 28 with virtual atresia) underwent RV decompression at median 3 (25th-75th, 2-5) days of age. Seventy-one patients (72%) underwent at least 1 reintervention after decompression. Median duration of follow-up was 3 years (range, 1-10). Freedom from reintervention was 51% at 1 month and 23% at 3 years. In multivariable analysis, reintervention was associated with virtual atresia (hazard ratio [HR], 0.51; 95% confidence interval [CI], 0.28-091; P=0.027), smaller RV length (HR, 0.94; 95% CI, 0.89-0.99; P=0.027), and ≤mild tricuspid regurgitation (TR; HR, 3.58; 95% CI, 2.04-6.30; P<0.001). Patients undergoing surgical shunt or ductal stent were less likely to have virtual atresia (HR, 0.36; 95% CI, 0.15-0.85; P=0.02) and more likely to have higher RV end-diastolic pressure (HR, 1.07; 95% CI, 1.00-1.15; P=0.057) and ≤mild TR (HR, 3.50; 95% CI, 1.75-7.0; P<0.001). Number of reinterventions was associated with ≤mild TR (rate ratio, 1.87; 95% CI, 1.23-2.87; P=0.0037). Multivariable analysis indicated that <2-ventricle circulation status was associated with ≤mild TR (odds ratio, 18.6; 95% CI, 5.3-65.2; P<0.001) and lower RV area (odds ratio, 0.81; 95% CI, 0.72-0.91; P<0.001).Patients with pulmonary atresia with intact ventricular septum deemed suitable for RV decompression have a high reintervention burden although most achieve 2-ventricle circulation. TR ≤mild at baseline is strongly associated with reintervention and <2-ventricle circulation at medium-term follow-up. Degree of baseline TR may be an important marker of long-term outcomes in this population.

    View details for DOI 10.1161/CIRCINTERVENTIONS.116.004428

    View details for Web of Science ID 000401546000004

    View details for PubMedID 28500137

  • Left ventricular rotational mechanics in early infancy: Normal reference ranges and reproducibility of peak values and time to peak values. Early human development Maskatia, S. A., Lee, W., Altman, C. A., Ayres, N. A., Feagin, D. K., Pignatelli, R. H. 2017; 104: 39-44


    Left ventricular cardiac twist and torsion values have been described in premature and term neonates, but not in early infancy. Early and late peak untwist rates and time to peak (TTP) values have not been described in infants.53 term infants were enrolled prospectively. The following parameters were obtained by two blinded observers at 1-2months postnatal age: peak twist and torsion (twist indexed to LV length), peak twist rate and torsion rate, TTP twist, early peak untwist rate, TTP early untwist rate, late peak untwist rate, TTP late untwist rate. Reproducibility was assessed using intraclass correlation and Bland Altman analysis.Intraclass correlation was ≥0.87 for all peak rotational mechanics values. Measures of TTP values had intraclass correlation (ICC) values ≤0.77, with TTP twist rate demonstrating the lowest ICC (0.69). The only measure which demonstrated significant bias was TTP twist rate. Peak twist demonstrated modest correlation (R=0.52, p<0.001) with global circumferential strain, and no correlation with ejection fraction, global longitudinal strain, or left ventricular myocardial performance index.Measurements of rotational mechanics and timing to peak values have acceptable reproducibility. Peak twist, twist rate, and early untwist rate values in early infancy are similar to those reported in premature neonates, and higher than those reported in older children. Twist indexed to LV length (torsion) is lower in early infancy than in premature neonates, but higher than in term neonates.

    View details for DOI 10.1016/j.earlhumdev.2016.11.004

    View details for PubMedID 28042971

  • Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy JOURNAL OF ULTRASOUND IN MEDICINE Ezon, D. S., Ayres, N. A., Altman, C. A., Denfield, S. W., Morris, S. A., Maskatia, S. A. 2016; 35 (9): 1949-1955


    Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes.We performed a 12-year retrospective review of fetuses with primary cardiomyopathy.Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0-36.4 weeks); median cardiovascular profile score was 6 (range, 1-9); median right ventricular Tei index was 0.52 (range, 0.32-0.94); and median left ventricular Tei index was 0.40 (range, 0.15-0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3-9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38-0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15-0.88; P = .50). Sixty-day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow-up of 39 months. Hydrops (P = .01), skin edema (P = .01), and mild or greater mitral regurgitation (P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation (P = .07) and fetal or postnatal demise.Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value.

    View details for DOI 10.7863/ultra.15.05059

    View details for PubMedID 27466259

  • Pilot study of chronic maternal hyperoxygenation and effect on aortic and mitral valve annular dimensions in fetuses with left heart hypoplasia ULTRASOUND IN OBSTETRICS & GYNECOLOGY Lara, D. A., Morris, S. A., Maskatia, S. A., Challman, M., Nguyen, M., FEAGIN, D. K., Schoppe, L., Zhang, J., Bhatt, A., Sexson-Tejtel, S. K., Lopez, K. N., Lawrence, E. J., Andreas, S., Wang, Y., Belfort, M. A., Ruano, R., Ayres, N. A., Altman, C. A., Aagaard, K. M., Becker, J. 2016; 48 (3): 365-372


    Acute maternal hyperoxygenation (AMH) results in increased fetal left heart blood flow. Our aim was to perform a pilot study to determine the safety, feasibility and direction and magnitude of effect of chronic maternal hyperoxygenation (CMH) on mitral and aortic valve annular dimensions in fetuses with left heart hypoplasia (LHH) after CMH.Gravidae with fetal LHH were eligible for inclusion in a prospective evaluation of CMH. LHH was defined as: sum of aortic and mitral valve annuli Z-scores < -4.5, arch flow reversal and left-to-right or bidirectional atrial level shunting without hypoplastic left heart syndrome or severe aortic stenosis. Gravidae with an affected fetus and with ≥ 10% increase in aortic/combined cardiac output flow after 10 min of AMH at 8 L/min 100% fraction of inspired oxygen were offered enrollment. Nine gravidae were enrolled from February 2014 to January 2015. The goal therapy was ≥ 8 h daily CMH from enrollment until delivery. Gravidae who were cared for from July 2012 to October 2014 with fetal LHH and no CMH were identified as historical controls (n = 9). Rates of growth in aortic and mitral annuli over the final trimester were compared between groups using longitudinal regression.There were no significant maternal or fetal complications in the CMH cohort. Mean gestational age at study initiation was 29.6 ± 3.2 weeks for the intervention group and 28.4 ± 1.8 weeks for controls (P = 0.35). Mean relative increase in aortic/combined cardiac output after AMH was 35.3% (range, 18.1-47.9%). Median number of hours per day on CMH therapy was 9.3 (range, 6.5-14.6) and median duration of CMH was 48 (range, 33-84) days. Mean mitral annular growth was 0.19 ± 0.05 mm/week compared with 0.14 ± 0.05 mm/week in CMH vs controls (mean difference 0.05 ± 0.05 mm/week, P = 0.33). Mean aortic annular growth was 0.14 ± 0.03 mm/week compared with 0.13 ± 0.03 mm/week in CMH vs controls (mean difference 0.01 ± 0.03 mm/week, P = 0.75). More than 9 h CMH daily (n = 6) was associated with better growth of the aortic annulus in intervention fetuses (0.16 ± 0.03 vs 0.08 ± 0.02 mm/week, P = 0.014).CMH is both safe and feasible for continued research. In this pilot study, the effect estimates of annular growth, using the studied method of delivery and dose of oxygen, were small. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.

    View details for DOI 10.1002/uog.15846

    View details for PubMedID 26700848

  • Fetal and Neonatal Diastolic Myocardial Strain Rate: Normal Reference Ranges and Reproducibility in a Prospective, Longitudinal Cohort of Pregnancies JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Pignatelli, R. H., Ayres, N. A., Altman, C. A., Sangi-Haghpeykar, H., Lee, W. 2016; 29 (7): 663-669


    Normative fetal diastolic strain rate values have not been comprehensively reported. The aims of this study were to report normative data for diastolic strain rate parameters across gestation and upon delivery, determine the effect of advancing gestational age on these measures, and compare interobserver variability at multiple gestational age time points.Sixty gravid women were enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and at 4 to 8 weeks' postnatal age: global left ventricular circumferential strain rate peak E and A waves, global left and right ventricular longitudinal strain rate peak E and A waves, and mitral and tricuspid valve E/A ratios. Reproducibility was assessed using intraclass correlation and Bland-Altman analysis. Least square means analysis was used to evaluate for changes across gestational age.Left ventricular longitudinal and circumferential diastolic strain rate values decreased across gestation, while right ventricular longitudinal values remained stable. With delivery, left ventricular values remained fairly stable, while right ventricular values increased. Intraclass correlations for diastolic strain rate values were 0.68 to 0.94 at ≥24 weeks' gestation and 0.25 to 0.82 for values at 20 to 21 weeks' gestation. Intraclass correlations ranged from 0.49 to 0.90 for mitral valve and tricuspid valve E/A ratios across gestation.Fetal measures of diastolic strain rate have acceptable reproducibility after 24 weeks' gestation. The described changes across gestation likely reflect intrinsic myocardial adaptation to loading conditions. These reference ranges can be used to assess effects of various disease states on fetal myocardial deformation.

    View details for DOI 10.1016/j.echo.2016.02.017

    View details for PubMedID 27049665

  • Association of Late Gadolinium Enhancement and Degree of Left Ventricular Hypertrophy Assessed on Cardiac Magnetic Resonance Imaging With Ventricular Tachycardia in Children With Hypertrophic Cardiomyopathy AMERICAN JOURNAL OF CARDIOLOGY Spinner, J. A., Noel, C. V., Denfield, S. W., Krishnamurthy, R., Jeewa, A., Dreyer, W. J., Maskatia, S. A. 2016; 117 (8): 1342-1348


    There are limited data on the clinical significance of left ventricular (LV) mass and late gadolinium enhancement (LGE) in pediatric hypertrophic cardiomyopathy (HC). We reviewed cardiovascular magnetic resonance (CMR) studies of children with HC to investigate the associations between the extent and distribution of LGE and LV mass with ventricular tachycardia (VT) in children with HC. A blinded observer reviewed CMR studies for the presence and distribution of LV hypertrophy and LGE using a 17-segment model. The primary outcome was VT. LGE was present 17 of 33 subjects (52%). VT was present on outpatient Holter monitor or exercise stress test in 7 patients, of which 5 patients (71%) had LGE. Each additional segment of LGE was associated with an increase in the odds of VT (odds ratio [OR] 1.4, 95% CI 1.1 to 1.9) and fewer than 5 segments with LGE had 93% specificity for the presence or absence of VT (OR 0.06, 95% CI 0.01 to 0.5). VT was more common in patients with LGE in the apical septal (p = 0.03), basal inferoseptal (p <0.01), and basal inferior (p = 0.04) segments, whereas LGE in more commonly involved segments (midanteroseptal and midinferoseptal) was not associated with VT (p = 0.13, 0.26). Patients with VT had greater LV mass index (76.4 ± 40.4 g/m(2.7) vs 50.9 ± 24.3 g/m(2.7); p = 0.03). Each centimeter of increased maximum LV thickness was associated with increased likelihood of VT (OR 2.9, 95% CI 1.2 to 6.8). In conclusion, in pediatric HC, CMR to evaluate the extent and pattern of LGE, LV mass index, and maximum LV thickness may help to identify children with HC at risk of VT.

    View details for DOI 10.1016/j.amjcard.2016.01.032

    View details for PubMedID 26892450

  • Longitudinal Changes and Interobserver Variability of Systolic Myocardial Deformation Values in a Prospective Cohort of Healthy Fetuses across Gestation and after Delivery JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Pignatelli, R. H., Ayres, N. A., Altman, C. A., Sangi-Haghpeykar, H., Lee, W. 2016; 29 (4): 341-349


    Normative data for fetal myocardial deformation values have not been comprehensively described in a longitudinal cohort. The effect of gestational age on these values and on interobserver variability require further investigation.Sixty gravid women were prospectively enrolled before 20 weeks' gestation. The following measures were obtained by two blinded observers at five time points across gestation and also at 4 to 8 weeks' postnatal age: global circumferential strain and strain rate, global longitudinal left ventricular strain and strain rate, global longitudinal right ventricular strain and strain rate, and left and right ventricular myocardial performance indices. Optimal myocardial visualization and frame rate (≥100 frames/sec) were ensured.For gestational age groups ≥24 weeks, intraclass correlation coefficients between observers were >0.70 for all measures and >0.85 for the majority of measures of myocardial deformation. At 20 to 21 weeks' gestation, intraclass correlation coefficients were 0.35 to 0.52 for longitudinal measures and 0.74 to 0.82 for circumferential measures. Myocardial performance index intraclass correlation coefficients were <0.80 at all time points and <0.70 for most time points. Global longitudinal left ventricular strain and global circumferential strain values remained stable across gestational age groups. Global longitudinal right ventricular strain values remained stable across gestation and increased after birth. Global circumferential strain rate, global longitudinal left ventricular strain rate, and global longitudinal right ventricular strain rate decreased from 20 to 21 weeks' gestation to the remainder of gestation and then remained stable until delivery. Upon delivery, global circumferential strain rate and global longitudinal left ventricular strain rate decreased, and global longitudinal right ventricular strain rate increased.Interobserver variability of fetal strain and strain rate measured at ≥24 weeks' gestation was lower in comparison with values obtained at 20 to 21 weeks' gestation and lower in comparison with left ventricular and right ventricular myocardial performance indices using the described protocol. Gestational changes in fetal myocardial deformation values likely reflect changes in preload and/or afterload on the fetal heart.

    View details for DOI 10.1016/j.echo.2015.12.003

    View details for PubMedID 26797677

  • Complex Surgical Repair of a Flail Tricuspid Valve After Chest Wall Trauma in a Pediatric Patient ANNALS OF THORACIC SURGERY Loar, R. W., Maskatia, S. A., McLaughlin, E. S., Mott, A. R., Adachi, I., Fraser, C. D. 2016; 101 (3): E65-E67


    Traumatic injury to the tricuspid valve can result from severe nonpenetrating chest wall trauma. We present the case of an initially asymptomatic 11-year-old girl who was kicked in the chest by a horse. The trauma resulted in avulsed papillary muscles, ruptured chordae, and right heart failure. She underwent early tricuspid valve reconstruction and annuloplasty. We advocate for routine use of echocardiography to assess for traumatic injury to intracardiac structures. In addition, we believe that early surgical intervention may prevent right-sided heart dysfunction, atrial arrhythmias, and the need for valve replacement.

    View details for DOI 10.1016/j.athoracsur.2015.09.041

    View details for PubMedID 26897232

  • Estimated Combined Cardiac Output Aids in the Assessment of Fetuses with Twin-Twin Transfusion Syndrome Echocardiography, In press Maskatia, S. 2016
  • Cardiovascular magnetic resonance techniques and findings in children with myocarditis: a multicenter retrospective study JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE Banka, P., Robinson, J. D., Uppu, S. C., Harris, M. A., Hasbani, K., Lai, W. W., Richmond, M. E., Fratz, S., Jain, S., Johnson, T. R., Maskatia, S. A., Lu, J. C., Samyn, M. M., Patton, D., Powell, A. J. 2015; 17


    Cardiovascular magnetic resonance (CMR) is increasingly used to diagnose myocarditis in adults but its use in children is not well-established. We sought to describe the presentation, CMR protocol and findings, and outcomes in a multicenter cohort of children with myocarditis.Thirteen hospitals retrospectively identified patients meeting the following inclusion criteria: 1) diagnosis of myocarditis by the managing physicians, 2) age <21 years, 3) CMR examination within 30 days of presentation, and 4) no congenital heart disease. Clinical data and test results, including CMR findings, were abstracted from the medical record.For the 143 patients meeting inclusion criteria, the median age was 16.0 years (range, 0.1-20.3) and 139 (97 %) were hospitalized at the time of CMR. The median time from presentation to CMR was 2 days (0-28). The median left ventricular ejection fraction at CMR was 56 % (10-74), with 29 (20 %) below 45 %. The median right ventricular ejection fraction was 54 % (15-72), with 11 (8 %) below 40 %. There was significant variability among centers in the types of tissue characterization techniques employed (p < 0.001). Overall, late gadolinium enhancement (LGE) was used in 100 % of studies, followed by T2-weighted imaging (T2W) in 69 %, first-pass contrast perfusion (FPP) in 48 %, and early gadolinium enhancement (EGE) in 28 %. Abnormalities were most common with LGE (81 %), followed by T2W (74 %), EGE (55 %), and FPP (8 %). The CMR study was interpreted as positive for myocarditis in 117 patients (82 %), negative in 18 (13 %), and equivocal in 7 (5 %), yielding a sensitivity of 82 %. At a median follow-up of 7.1 months (0-87), all patients were alive and 5 had undergone cardiac transplantation. CMR parameters at presentation associated with persistent left ventricular dysfunction were larger left ventricular end-diastolic volume and lower left and right ventricular ejection fraction but not abnormal LGE.Despite significant practice variation in imaging protocol among centers, CMR had a high sensitivity for the diagnosis of myocarditis in pediatric patients. Abnormalities were most often seen with LGE followed by T2W, EGE, and FPP. These findings should be useful in designing future prospective studies.

    View details for DOI 10.1186/s12968-015-0201-6

    View details for PubMedID 26576638

  • Older Age at Completion of Fontan Procedure Is Associated with Improved Percentage of Predicted Maximum Oxygen Uptake TEXAS HEART INSTITUTE JOURNAL Bolin, E. H., Maskatia, S. A., Tate, A. L., Petit, C. J. 2015; 42 (4): 333-340


    We tested the hypothesis that later completion of the Fontan procedure is associated with improved exercise capacity in the current period of staged single-ventricle palliation. We performed a retrospective study, in Fontan patients, of exercise stress test data from April 2003 through March 2011. Patients were included if they had received staged palliations in accordance with current surgical strategy, defined as the performance of a superior cavopulmonary connection at ≤1 year of age, followed in subsequent years by Fontan completion. Patients with a pacemaker or respiratory exchange ratio <1 were excluded. Early and late Fontan groups were created on the basis of whether Fontan completion had been performed at <4 or ≥ 4 years of age. The primary predictor variable was age at Fontan completion, and the primary marker of exercise performance was the percentage of predicted maximum oxygen consumption. During the study period, 55 patients were identified (mean age, 11.7 ± 2.8 yr). Older age at Fontan completion correlated positively with higher percentages of predicted maximum oxygen consumption (R=0.286, P=0.034). Patients in whom Fontan completion was performed at ≥4 years of age had higher percentages of predicted maximum oxygen consumption than did those in whom completion was at <4 years of age (84.4 ± 21.5 vs 72.9 ± 18.1; P=0.041). Later Fontan completion might be associated with improved exercise capacity in patients palliated in accordance with contemporary surgical strategy.

    View details for DOI 10.14503/THIJ-14-4178

    View details for Web of Science ID 000360211900007

    View details for PubMedID 26413015

  • Congenital heart anomaly in newborns with congenital diaphragmatic hernia: a single-center experience ULTRASOUND IN OBSTETRICS & GYNECOLOGY Ruano, R., Javadian, P., Kailin, J. A., Maskatia, S. A., Shamshirsaz, A. A., Cass, D. L., Zamora, I. J., Sangi-Haghpeykar, H., Lee, T. C., Ayres, N. A., Mehollin-Ray, A., Cassady, C. I., Fernandes, C., Welty, S., Belfort, M. A., Olutoye, O. O. 2015; 45 (6): 683-688


    To evaluate the impact of the presence of a congenital heart anomaly (CHA) and its potential contribution to morbidity and mortality in infants with congenital diaphragmatic hernia (CDH).In this retrospective cohort study, prenatal and postnatal data of all newborns diagnosed with CDH between January 2004 and December 2012 in a single center were reviewed. Cases were classified into two groups: those with 'isolated' CDH and those with both CDH and CHA. Patients with CHA were further subclassified into those with a major or minor CHA based on the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and the Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STS-EACTS) scoring systems. Patients with associated non-cardiac anomalies, including 'syndromic cases', were excluded from the analysis. Primary and secondary outcomes were survival up to 1 year of age and a need for extracorporeal membrane oxygenation (ECMO), respectively.Of the 180 infants with CDH, 41 were excluded because of the presence of non-cardiac associated anomalies, 118 had isolated CDH and 21 had CDH with CHA (16 with minor and five with major CHA). Receiver-operating characteristics curve analysis demonstrated that the best cut-off for survival was when the score for CHA was ≤ 2 for both RACHS-1 (area under the curve (AUC), 0.74 (P = 0.04); sensitivity, 80.0%; specificity, 87.5%) and STS-EACTS (AUC, 0.83 (P = 0.03); sensitivity, 100%; specificity, 87.5%). Survival rate at 1 year was significantly lower in those with CHD and a major CHA (40.0%; P = 0.04) than in those with isolated CDH (77.1%) and those with CDH and a minor CHA (81.3%). We found no significant differences among the groups with regard to the need for ECMO.In general, a milder form of CHA does not appear to have a negative impact on the survival of infants with CDH. However, mortality appears to be significantly higher in infants with CDH and a major form of CHA. The scoring systems appear to be useful as predictors for classifying the effects of CHA in this population of patients.

    View details for DOI 10.1002/uog.14648

    View details for Web of Science ID 000355674700011

    View details for PubMedID 25158239

  • Echocardiographic Parameters of Right Ventricular Diastolic Function in Repaired Tetralogy of Fallot Are Associated with Important Findings on Magnetic Resonance Imaging CONGENITAL HEART DISEASE Maskatia, S. A., Morris, S. A., Spinner, J. A., Krishnamurthy, R., Altman, C. A. 2015; 10 (3): E113-E122


    Right ventricular (RV) dilation, RV dysfunction, RV outflow tract obstruction, and branch pulmonary artery stenosis are risk factors associated with the need for future surgical or catheter-based intervention commonly assessed by cardiac magnetic resonance in patients with tetralogy of Fallot who have undergone initial repair. The ability of echocardiography to predict the presence of these risk factors is limited.We aimed to determine the association between echocardiographic parameters of right ventricular diastolic dysfunction and risk factors for intervention as identified on cardiac magnetic resonance imaging (CMR).We reviewed all echocardiograms and CMRs performed at our institution within 1 year of each other in patients with repaired tetralogy of Fallot. We evaluated patients for the following: RV systolic dysfunction (RV ejection fraction <45%), RV dilation (right ventricular end-diastolic volume ≥ 160 mL/m(2)), and RV obstruction (RV outflow tract gradient ≥ 3 m/s by echocardiogram or branch pulmonary artery stenosis assessed by CMR). We evaluated for associations between the above CMR findings and echocardiographic parameters of diastolic function.CMR and echocardiographic images performed between March 2007 and March 2012 were available in 99 patients. RV obstruction was associated with lower E/A ratio (P = .02), E/A reversal (P = .02), and prograde end diastolic pulmonary flow (P < .01). Low tricuspid annular a' and low septal s' were associated with lower RV ejection fraction (P < .01, P < .01). Elevated E/e' was associated with higher RV end diastolic volume (P = .04). In predicting the presence of any of the evaluated risk factors for future intervention, TV E/e' ≥ 4.7 had positive predictive value (PPV) of 86% and negative predictive value (NPV) of 50%, TV E/A reversal had PPV of 89% and NPV of 34, and prograde end diastolic pulmonary flow on echocardiogram had PPV of 91% and NPV of 38%.Echocardiographic parameters of diastolic dysfunction have a reasonable PPV but poor NPV for predicting clinically significant risk factors identified by CMR.

    View details for DOI 10.1111/chd.12265

    View details for PubMedID 25916551

  • Clinical validation of free breathing respiratory triggered retrospectively cardiac gated cine balanced steady-state free precession cardiovascular magnetic resonance in sedated children JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE Krishnamurthy, R., Pednekar, A., Atweh, L. A., Vogelius, E., Chu, Z. D., Zhang, W., Maskatia, S., Masand, P., Morris, S. A., Krishnamurthy, R., Muthupillai, R. 2015; 17


    Cine balanced steady-state free precession (SSFP), the preferred sequence for ventricular function, demands uninterrupted radio frequency (RF) excitation to maintain the steady-state during suspended respiration. This is difficult to accomplish in sedated children. In this work, we validate a respiratory triggered (RT) SSFP sequence that drives the magnetization to steady-state before commencing retrospectively cardiac gated cine acquisition in a sedated pediatric population.This prospective study was performed on 20 sedated children with congenital heart disease (8.6 ± 4 yrs). Identical imaging parameters were used for multiple number of signal averages (MN) and RT cine SSFP sequences covering both the ventricles in short-axis (SA) orientation. Image quality assessment and quantitative volumetric analysis was performed on the datasets by two blinded observers. One-sided Wilcoxon signed rank test and Box plot analysis were performed to compare the clinical scores. Bland-Altman (BA) analysis was performed on LV and RV volumes.Scan duration for SA stack using RT-SSFP (3.9 ± 0.8 min) was slightly shorter than MN-SSFP (4.6 ± 0.9 min) acquisitions. The endocardial edge definition was significantly better for RT than MN, blood to myocardial contrast was better for RT than MN without reaching statistical significance, and inter slice alignment was comparable. BA analysis indicates that the variability of volumetric indices between RT and MN is comparable to inter and intra-observer variability reported in the literature.The free breathing RT-SSFP sequence allows diagnostic images in sedated children with significantly better edge definition when compared to MN-SSFP, without any penalty for total scan time.

    View details for DOI 10.1186/s12968-014-0101-1

    View details for Web of Science ID 000349945400001

    View details for PubMedID 25589308

  • Marked Septal Dyskinesis From Wolff-Parkinson-White Syndrome CIRCULATION Niu, M. C., Maskatia, S. A., Kim, J. J. 2014; 130 (23): E196-E198
  • Surgical management of tetralogy of fallot: in defense of the infundibulum. Seminars in thoracic and cardiovascular surgery McKenzie, E. D., Maskatia, S. A., Mery, C. 2013; 25 (3): 206-212


    Surgical treatment of the Tetralogy of Fallot (ToF) is one of the great successes of medicine and also a topic of controversy. Different strategies have been proposed, including age-based (neonatal) management strategies as well as anatomic-based management strategies. Regardless of the management strategy entailed, the surgical management of ToF has considerably evolved over the years. As a result, patients can now expect excellent early results with survival approaching 100% for those without genetic syndromes. The goals of current surgical therapy should be to mitigate the late right ventricular (RV) dysfunction that may occur by minimizing the extent of surgical injury during the intial repair. As the surgical techniques continue to advance, the outcomes will continue to improve.

    View details for DOI 10.1053/j.semtcvs.2013.10.003

    View details for PubMedID 24331142

  • Repeat Balloon Aortic Valvuloplasty Effectively Delays Surgical Intervention in Children With Recurrent Aortic Stenosis CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Petit, C. J., Maskatia, S. A., Justino, H., Mattamal, R. J., Crystal, M. A., Ing, F. F. 2013; 82 (4): 549-555


    Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Recurrent AS following initial BAV or initial surgical valvotomy (SV) may require a second BAV (BAV2). We sought to determine the longterm outcomes of BAV2.We reviewed all cases of BAV2, defined as BAV following primary BAV or SV between 1988 and 2009. Cases were reviewed for pre- and post-BAV2 echocardiographic and procedural details.Tertiary care dedicated children's hospital.Between 1985 and 2009, 43 patients underwent BAV2 (23 primary SV, 20 primary BAV) at median age 1.9 years (1 month-21 years) and median weight 15 (3.3-55) kg.BAV2 performed following primary SV or primary BAV.We evaluated the following endpoints: ≥ moderate AI post-BAV2, aortic valve replacement (AVR), additional BAV or SV post-BAV2, death and heart transplantation.The gradient decreased from 61.4 ± 16.0 mm Hg to 26.0 ± 13.6 post-BAV2 (P < 0.01). Gradient prior to BAV2 was higher in primary SV patients (66 ± 13 mm Hg) than in primary BAV patients (56 ± 18 mm Hg, P = 0.04). 24 patients had no further events after BAV2, while 19 patients (44%) experienced 23 events including: AVR (n = 8), SV (n = 6), BAV3 (n = 2), death (n = 5), and transplant (n = 1). Regression demonstrated that adverse events were associated with higher post-BAV2 gradient (P < 0.01). Repeat intervention on the aortic valve and AVR were associated with higher post BAV2 gradient (P = 0.04, P = 0.01). Prior to BAV2, 7 patients (17%) had AI > mild, compared to 21 (51%) patients after BAV2. Cox regression revealed that primary BAV was associated with development of AI > mild after BAV2 (P < 0.01).BAV2 is associated with decreased valve gradient, though with an increase in AI. However, residual AS, not AI, is associated with poor outcomes following BAV2. BAV2 effectively treats recurrent AS and postpones need for surgical intervention.

    View details for DOI 10.1002/ccd.24562

    View details for Web of Science ID 000329285200036

    View details for PubMedID 22815228

  • The Echocardiography "Boot Camp": A Novel Approach in Pediatric Cardiovascular Imaging Education JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Maskatia, S. A., Altman, C. A., Morris, S. A., Cabrera, A. G. 2013; 26 (10): 1187-1192


    Dynamic training schedules introduce novel challenges to medical specialty training programs that require manual dexterity. The aim of this study was to examine the effect of a 3-day, intensive pediatric echocardiography course ("boot camp") on trainee self-efficacy and on the acquisition and short-term retention of basic echocardiographic knowledge and skills for first-year pediatric cardiology fellows (CFs).The boot camp consisted of hands-on structured practice guided by sonographers and cardiology faculty members, didactic lectures, and reading. Pre-boot camp experience was assessed using an experience score. Outcome measures included written precamp and postcamp examinations, a performance-based test, precamp and postcamp self-efficacy assessments, and the number and quality of echocardiographic examinations performed in the first 3 months of fellowship.Six CFs completed the boot camp. Two of the six CFs reported experience scores of 2 out of 10, whereas the remainder reported experience scores of 0 out of 10. Performance-based test scores ranged from 68 to 99 out of 147. All six CFs reported precamp self-efficacy scores of 21 (the minimum score), compared with median postcamp scores of 82 (range, 49-94) (P = .01). Scores on the written examination improved from median of 16 (range, 11-18) to 23.5 (range, 22-28) (P = .01). CFs who completed the boot camp completed 28 independent echocardiographic examinations (median, 4 per CF) during the first 3 months of fellowship, an increase from six independent examinations (median, 1 per CF) by CFs during the year before institution of the boot camp (P = .030). Echocardiograms obtained by CFs who had completed the boot camp scored higher on total quality (P = .004), overall two-dimensional image quality (P = .011), functional assessments (P = .015), and assessment for pericardial effusion (P = .031).The echocardiography boot camp improves self-efficacy in performing an echocardiographic examination and the acquisition and short-term retention of skills and knowledge required to perform pediatric echocardiography.

    View details for DOI 10.1016/j.echo.2013.06.001

    View details for Web of Science ID 000324748300011

    View details for PubMedID 23860091

  • Impact of Obesity on Ventricular Size and Function in Children, Adolescents and Adults With Tetralogy of Fallot After Initial Repair AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Spinner, J. A., Nutting, A. C., Slesnick, T. C., Krishnamurthy, R., Morris, S. A. 2013; 112 (4): 594-598


    Obesity is epidemic in congenital heart disease, with reported rates of 16% to 26% in children and 54% in adults. The aim of this study was to evaluate the impact of obesity on ventricular function and size in patients after initial repair for tetralogy of Fallot (TOF). Cardiac magnetic resonance studies in normal-weight (body mass index percentile <85th) and obese (body mass index percentile ≥95th) children and adults with repaired tetralogy of Fallot were reviewed. The left ventricular ejection fraction, the right ventricular ejection fraction, left and right ventricular end-diastolic volumes indexed to actual body surface area, to height, and to body surface area using ideal body weight were evaluated in 36 obese patients and 72 age-matched normal-weight patients. Compared with normal-weight patients, obese patients had lower right ventricular ejection fractions (mean 46 ± 9% vs 51 ± 7%, p = 0.003) and left ventricular ejection fractions (mean 57 ± 9% vs 61 ± 6%, p = 0.017), higher right ventricular end-diastolic volumes indexed to height (mean 160 ± 59 vs 135 ± 41 ml/m, p = 0.015) and left ventricular end-diastolic volumes indexed to height (mean 86 ± 25 vs 70 ± 20 ml/m, p = 0.001), and higher right ventricular end-diastolic volumes indexed to ideal body weight (mean 166 ± 55 vs 144 ± 38 ml/m², p = 0.020) and left ventricular end-diastolic volumes indexed to ideal body weight (mean 90 ± 22 vs 75 ± 15 ml/m², p <0.001). In conclusion, obesity is a modifiable risk factor associated with worsened biventricular systolic function and biventricular dilation in patients with repaired tetralogy of Fallot. The standard method of indexing ventricular volumes using actual body surface area may underestimate volume load in obese patients.

    View details for DOI 10.1016/j.amjcard.2013.04.030

    View details for Web of Science ID 000323407700023

    View details for PubMedID 23677064

  • Effect of Branch Pulmonary Artery Stenosis on Right Ventricular Volume Overload in Patients With Tetralogy of Fallot After Initial Surgical Repair AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Spinner, J. A., Morris, S. A., Petit, C. J., Krishnamurthy, R., Nutting, A. C. 2013; 111 (9): 1355-1360


    Right ventricular (RV) volume overload secondary to pulmonary regurgitation is common in patients after initial repair of tetralogy of Fallot (TOF) and is associated with adverse long-term outcomes. The objective of the present study was to determine the effect of branch pulmonary artery stenosis on the RV volume in patients with repaired TOF. We reviewed 178 cardiac magnetic resonance imaging studies in patients with repaired TOF. We defined bilateral stenosis as a Nakata index of ≤200 mm(2)/m(2) and concordant branch pulmonary artery cross-sectional area, unilateral stenosis as 1 branch pulmonary artery cross-sectional area ≤100 mm(2)/m(2) and 1 branch pulmonary artery cross-sectional area >100 mm(2)/m(2), and restrictive physiology as prograde main pulmonary artery diastolic flow. Of the 178 patients, 20 (11%) had bilateral stenosis, 47 (26%) unilateral stenosis, and 111 (63%) had no stenosis. The RV end-diastolic volume was lower in patients with bilateral (125 ± 27 ml/m(2)) or unilateral (131 ± 43 ml/m(2)) stenosis than in those without stenosis (149 ± 35 ml/m(2), p = 0.021 and p = 0.019, respectively). The main pulmonary artery regurgitant fraction was greater in patients without stenosis (47%, range 2% to 69%) than in those with bilateral (33%, range 9% to 59%; p = 0.009) or unilateral stenosis (40%, range 0% to 71%; p = 0.033). Restrictive physiology was more common in patients with bilateral (13 of 15, 87%) or unilateral (21 of 38, 55%) stenosis than in those without stenosis (28 of 85, 33%; p <0.001 and p = 0.017, respectively). In conclusion, in patients with repaired TOF, bilateral and unilateral branch pulmonary artery stenosis was associated with a greater main pulmonary artery regurgitant fraction and smaller RV end-diastolic volume than those in patients without stenosis, likely owing to the development of restrictive physiology. Branch pulmonary artery stenosis might effectively delay the referral for pulmonary valve replacement.

    View details for DOI 10.1016/j.amjcard.2013.01.278

    View details for Web of Science ID 000318827900020

    View details for PubMedID 23411101

  • Aortic valve morphology is associated with outcomes following balloon valvuloplasty for congenital aortic stenosis CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Maskatia, S. A., Justino, H., Ing, F. F., Crystal, M. A., Mattamal, R. J., Petit, C. J. 2013; 81 (1): 90-95


    Evaluate the incidence of various morphologic types of congenital AS, and the association between valve morphology and long-term outcomes, including repeat BAV, AVR, and death/transplant.Reports on long-term outcomes have low agreement on the influence of morphologic type.We queried our institutional database and hospital billing records to identify all patients who underwent balloon aortic valvuloplasty (BAV) from 1992 through 2009. We excluded cases where morphology was not clear based on the description in the pre-BAV echocardiogram report and patients who underwent single ventricle palliation. The primary outcome of the study was the occurrence of any of the following events: repeat valvuloplasty, AVR, heart transplant, or death.There were 147 patients in our study cohort. The most common morphology was functionally bicuspid (n = 92, 63%), followed by functionally unicuspid (n = 20, 14%), dysplastic (n = 16, 11%), true bicuspid (n = 13, 9%), and true unicuspid (n = 6, 4%). The primary endpoint was less likely to occur in patients with functionally bicuspid valves (P < 0.01) and patients with true bicuspid valves (P = 0.03), whereas it was more likely to occur in patients with functionally unicuspid valves (P = 0.02) and patients with true unicuspid valves (P = 0.05). Multivariate Cox regression analysis demonstrated that valve type other than functionally bicuspid was associated with diminished freedom from repeat intervention, death or transplant (HR 3.3, CI 1.2 - 8.6, P = 0.02).In our cohort, patients with functionally bicuspid aortic valves, the most common type, had improved outcomes as compared with all other morphologic types.

    View details for DOI 10.1002/ccd.24286

    View details for Web of Science ID 000312942300033

    View details for PubMedID 23044747

  • Morphologic Heterogeneity in Left Ventricular Noncompaction Resulting in Accessory Left Ventricular Chambers CIRCULATION-HEART FAILURE Ezon, D., Maskatia, S., Jeewa, A., Denfield, S. 2012; 5 (6): E94-E95
  • Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maskatia, S. A., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. 2012; 144 (1): 184-189


    The aims of our study are to describe the incidence, clinical profile, and risk factors for pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. We hypothesized the following: (1) Pulmonary reperfusion injury is more likely to occur after unifocalization procedures in which a septated circulation is not achieved, (2) pulmonary reperfusion injury is directly related to the severity of stenosis in major aortopulmonary collateral arteries, and (3) pulmonary reperfusion injury leads to longer intubation time and longer hospitalization.Consecutive patients with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries who underwent unifocalization procedures over a 5-year period were identified in our institutional database. Chest radiographs before the unifocalization procedure, from postoperative days 0 to 4, and from 2 weeks after the unifocalization procedure or at discharge were evaluated by a pediatric radiologist for localized pulmonary edema. Determination of stenosis severity was based on review of preoperative angiograms. Statistical analyses using multivariate repeated-measures analyses were performed with generalized estimating equations.Pulmonary reperfusion injury was present after 42 of 65 (65%) unifocalization procedures. In 36 of 42 cases of reperfusion injury, unilateral injury was present. Risk factors for the development of reperfusion injury included bilateral unifocalization (P = .01) and degree of stenosis (P = .03). We did not identify an association between pulmonary reperfusion injury and time to tracheal extubation or hospital discharge.Pulmonary reperfusion injury is common after the unifocalization procedure for tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries. Severity of stenosis and bilateral unifocalization are associated with the development of reperfusion injury.

    View details for DOI 10.1016/j.jtcvs.2011.12.030

    View details for Web of Science ID 000305412200031

    View details for PubMedID 22244564

  • Type B Interrupted Left Aortic Arch with Isolated Right Subclavian Artery CONGENITAL HEART DISEASE Knudson, J. D., Lopez, K. N., Maskatia, S., McKenzie, E. D., Lantin-Hermoso, M. R., Masand, P. M., Vick, G. W. 2012; 7 (3): E25-E30


    Interrupted aortic arch is a rare congenital heart malformation occurring in approximately three per 1 million births. Type B interrupted aortic arch (interruption between the second carotid artery and the ipsilateral subclavian artery) is the most common of three major types (A, B, and C). We report an extremely rare finding: a case of left-sided type B interrupted aortic arch with isolation of the right subclavian artery (origin from the right pulmonary artery).

    View details for DOI 10.1111/j.1747-0803.2011.00625.x

    View details for Web of Science ID 000304437100007

    View details for PubMedID 22309184

  • Hypertrophic Cardiomyopathy: Infants, Children, and Adolescents CONGENITAL HEART DISEASE Maskatia, S. A. 2012; 7 (1): 84-92


    Hypertrophic cardiomyopathy (HCM) is characterized by inappropriate left ventricular hypertrophy (LVH) in the setting of a nondilated left ventricle. HCM is often associated with asymmetric LVH, a family history of HCM, sarcomeric genetic mutations, and an increased risk of sudden cardiac death. There is a wide clinical variability in HCM presenting during childhood and a relative lack of data on the pediatric population. This review will cover HCM presenting in infancy, childhood, and adolescence.

    View details for DOI 10.1111/j.1747-0803.2011.00613.x

    View details for Web of Science ID 000299466700016

    View details for PubMedID 22222117

  • Restrictive Physiology is Associated With Poor Outcomes in Children With Hypertrophic Cardiomyopathy PEDIATRIC CARDIOLOGY Maskatia, S. A., Decker, J. A., Spinner, J. A., Kim, J. J., Price, J. F., Jefferies, J. L., Dreyer, W. J., Smith, E. O., Rossano, J. W., Denfield, S. W. 2012; 33 (1): 141-149


    Pediatric patients with hypertrophic cardiomyopathy (HCM) and restrictive physiology (RP) with poor outcomes have been identified, but data on their course are limited. Our goal was to delineate the clinical features and course of children with HCM and RP. An institutional review of 119 patients identified between 1985 and 2010 with the diagnosis of HCM was performed. The diagnosis of RP was based on >1 echocardiogram along with at least one of the following: left atrial enlargement without evidence of left ventricle dilation, E/E' ratio ≥ 10, and E/A ratio ≥ 3. Outcomes analysis was performed using Cox or Poisson regression when appropriate. RP was present in 50 (42%) patients. In patients without RP, 10-year freedom-from-death or aborted sudden cardiac death (aSCD), and death or heart transplant (HT), were 93.6 and 98.5%, respectively. In patients with RP, 10-year freedom-from-death or aSCD, and death or HT, were 59.0 and 71.2%, respectively. RP conferred a 3.5-fold increase in incidence rate of hospitalization (P = 0.01), a 3.8-fold increase in hazard of death or aSCD (P = 0.02), and a 5.7-fold increase in hazard of death or HT (P = 0.04). Assessment for RP is of paramount importance in children with HCM because those without RP have a good prognosis, and those with RP account for the majority of poor outcomes.

    View details for DOI 10.1007/s00246-011-0106-6

    View details for Web of Science ID 000298660100021

    View details for PubMedID 21892651

  • Twenty-Five Year Experience With Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis AMERICAN JOURNAL OF CARDIOLOGY Maskatia, S. A., Ing, F. F., Justino, H., Crystal, M. A., Mullins, C. E., Mattamal, R. J., Smith, E. O., Petit, C. J. 2011; 108 (7): 1024-1028


    Balloon aortic valvuloplasty (BAV) is the primary therapy for congenital aortic stenosis (AS). Few reports describe long-term outcomes. In this study, a retrospective single-institution review was performed of patients who underwent BAV for congenital AS. The following end points were evaluated: moderate or severe aortic insufficiency (AI) by echocardiography, aortic valve replacement, repeat BAV, surgical aortic valvotomy, and transplantation or death. From 1985 to 2009, 272 patients who underwent BAV at ages 1 day to 30.5 years were followed for 5.8 ± 6.7 years. Transplantation or death occurred in 24 patients (9%) and was associated with depressed baseline left ventricular shortening fraction (LVSF) (p = 0.04). Aortic valve replacement occurred in 42 patients (15%) at a median of 3.5 years (interquartile range 75 days to 5.9 years) after BAV and was associated with post-BAV gradient ≥25 mm Hg (p = 0.02), the presence of post-BAV AI (p = 0.03), and below-average baseline LVSF (p = 0.04). AI was found in 83 patients (31%) at a median of 4.8 years (interquartile range 1.4 to 8.7) and was inversely related to post-BAV gradient ≥25 mm Hg (p <0.04). AI was associated with depressed baseline LVSF (p = 0.02). Repeat valvuloplasty (balloon or surgical) occurred in 37 patients (15%) at a median of 0.51 years (interquartile range 0.10 to 5.15) and was associated with neonatal BAV (p <0.01), post-BAV gradient ≥25 mm Hg (p = 0.03), and depressed baseline LVSF (p = 0.05). In conclusion, BAV confers long-term benefits to most patients with congenital AS. Neonates, patients with post-BAV gradients ≥25 mm Hg, and patients with lower baseline LVSF experienced worse outcomes.

    View details for DOI 10.1016/j.amjcard.2011.05.040

    View details for Web of Science ID 000295863200022

    View details for PubMedID 21791328

  • Congenital Anomalies of the Mitral Valve CONGENITAL HEART DISEASE Maskatia, S. A. 2011; 6 (1): 77-82