Treatment Strategies and Outcomes in Pulmonary Atresia With Ventricular Septal Defect Without Major Aortopulmonary Collateral Arteries in North America 2003-2023.

Abstract

The management of pulmonary atresia with ventricular septal defect (PA/VSD) without major aortopulmonary collateral arteries (MAPCAs) varies widely, yet multicenter outcome data are limited. This study employed a multi-institutional database to explore practice patterns, treatment strategies, and outcomes. We identified 304 patients with PA/VSD without MAPCAs from the Pediatric Health Information System database who underwent biventricular repair between January 1, 2003, and December 31, 2023. We compared in-hospital mortality, postoperative outcomes, and 1-year and 2-year mortality rates of patients receiving single-stage early complete repair, patent ductus arteriosus (PDA) stent, systemic-to-pulmonary artery shunt, or right ventricle-to-pulmonary artery (RV-PA) connection. Temporal trends in management strategies across 4 eras were also assessed. Of the 304 patients, 135 underwent single-stage complete repair, 41 received a PDA stent, 97 had a systemic-to-pulmonary artery shunt, and 31 had an RV-PA connection. In-hospital mortality after initial palliation was lower with systemic-to-pulmonary artery shunt than with early complete repair (2.1% vs 11.8%; P = .03). In-hospital mortality at complete repair was 9.1% (P = .04) for PDA stent, 4.8% (P = .4) for RV-PA connection, and no death in systemic-to-pulmonary artery shunt groups. The 2-year cumulative mortality was comparable between the groups, with Kaplan-Meier survival analysis showing no survival differences. Early complete repair of PA/VSD without MAPCAs is gaining prominence and yields short-term and midterm outcomes comparable to staged approaches.