1. Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi-institutional study.
- Author
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Joong A, Zuckerman WA, Koehl D, Cantor R, Alejos JC, Ameduri RK, Boyle GJ, Rothkopf AC, Kirklin JK, and Gajarski RJ
- Subjects
- Child, Humans, Infant, Retrospective Studies, Treatment Outcome, Heart Defects, Congenital, Heart Transplantation, Pulmonary Atresia surgery, Ventricular Septum
- Abstract
Background: Management of infants with pulmonary atresia/intact ventricular septum (PA/IVS) is variable. Because of higher mortality in more severe forms, heart transplant (HT) is an acceptable approach, but waitlist and post-transplant outcomes are unclear. This study compared outcomes of infants with PA/IVS vs. other single ventricle (SV) anatomies listed for HT., Methods: Data from the Pediatric Heart Transplant Society (1993-2018) were analyzed for survival and risk factors for mortality., Results: Of 1617 SV infants, 300 had PA/IVS (19%) and 1317 had other SV (81%). Overall, 1-, 5-, and 10-year survival was higher among PA/IVS (74%, 65%, 61%) versus other SV infants (62%, 54%, 50%, p = .004). While waitlist mortality was similar between groups (p = .09), PA/IVS was an independent predictor of improved waitlist survival (HR 0.68, p = .03), and PA/IVS infants had higher incidence of waitlist removal (8% vs. 5.5%, p = .03), most commonly for being "too well." Post-transplant survival was superior among PA/IVS versus other SV infants (1- and 5-year survival 93% and 81% vs. 80% and 71%, pā<ā.0001). Risk factors for PA/IVS waitlist mortality (2008-2018) included extracorporeal membrane oxygenation and mechanical ventilation. Prior aortopulmonary (AP) shunt among PA/IVS infants was associated with improved waitlist survival., Conclusions: Overall survival among PA/IVS infants listed for HT exceeds that of other SV infants with PA/IVS identified as an independent predictor of improved waitlist and post-transplant survival. Prior AP shunt among listed PA/IVS infants was associated with improved waitlist outcomes, though, which may reflect a listing selection bias., (© 2022 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.)
- Published
- 2022
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