Treatment Strategies and Outcomes in Pulmonary Atresia with Ventricular Septal Defect without Major Aorto-pulmonary Collateral Arteries in North America 2003-2023.

Background: The management of Pulmonary Atresia with Ventricular Septal Defect (PA/VSD) without Major Aortopulmonary Collaterals (MAPCAs) varies widely, yet multicenter outcome data is limited. This study employs a multi-institutional database to explore practice patterns, treatment strategies, and outcomes.

Methods: 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 among patients receiving single-stage early complete repair, patent ductus arteriosus stent, systemic-to-pulmonary artery shunt, or right ventricle-to-pulmonary artery connection. Temporal trends in management strategies across four eras were also assessed.

Results: Of the 304 patients, 135 underwent single-stage complete repair, 41 received a patent ductus arteriosus stent, 97 had a systemic-to-pulmonary artery shunt, and 31 had a right-ventricle to pulmonary artery connection. In-hospital mortality following initial palliation was lower with systemic-to-pulmonary artery shunt than with early complete repair (2.1% vs 11.8%, p = 0.03). In-hospital mortality at complete repair was 9.1% (p=0.04) for patent ductus arteriosus stent, 4.8% (p=0.4) for right-ventricle to pulmonary artery connection, and no death in systemic-to-pulmonary artery shunt groups. 2-year cumulative mortality was comparable between the groups with Kaplan-Meier survival analysis showing no survival differences.

Conclusions: Early complete repair of PA/VSD without MAPCAs is gaining prominence and yields short- and mid-term outcomes comparable to staged approaches.