Neonatal Primary Repair of Pulmonary Atresia with Ventricular Septal Defect without Major Aortopulmonary Collateral Arteries: midterm Outcomes

OBJECTIVES Pulmonary atresia with ventricular septal defect (PA-VSD) without major aortopulmonary collateral arteries is a rare congenital heart defect. Surgical management strategies include staged palliation or primary neonatal repair. We evaluated the midterm outcomes of neonatal primary repair in patients without MAPCAs. METHODS A retrospective single-center analysis was performed of 31 neonates who underwent primary repair for PA-VSD without MAPCAs between 2005 and 2024. Primary outcomes included mortality, reintervention rates, and right ventricular (RV) function. RESULTS Thirteen patients (41.9%) underwent urgent surgery and 18 (58.1%) underwent emergent surgery. Median weight at repair was 2.8 kg (interquartile range [IQR] 2.6–3.2). Overall survival was 96.8% (n = 30), with one mortality (3.2%) related to multi-organ failure. Postoperative RV function was normal in 25 patients (80.7%). Median follow-up for hospital survivors was 5.4 years (IQR 1.2–7.6). Freedom from surgical reoperation was 56.7%; 13 patients (43.3%) required reoperation, most commonly for RV-to-pulmonary artery conduit revision (n = 11). Catheter-based reinterventions were performed in 24 patients (80.0%). RV function remained preserved in 29 patients (96.7%) at last follow-up. CONCLUSIONS Neonatal primary repair of PA-VSD without major aortopulmonary collaterals can be performed with excellent midterm survival and preserved right ventricular function. Reintervention, largely conduit-related, represents an anticipated component of longitudinal management rather than failure of the primary strategy.