Outcomes of infants with pulmonary atresia with intact ventricular septum listed for heart transplantation: A multi‐institutional study

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%, p