Neonatal Primary Repair of Pulmonary Atresia With Ventricular Septal Defect Without Major Aortopulmonary Collateral...

Pulmonary atresia with ventricular septal defect (PA-VSD) without major aortopulmonary collateral arteries (MAPCAs) 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.

A retrospective single-centre 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.

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%). Right ventricular function remained preserved in 29 patients (96.7%) at last follow-up.

Neonatal primary repair of PA-VSD without major aortopulmonary collaterals can be performed with excellent midterm survival and preserved RV function. Reintervention, largely conduit-related, represents an anticipated component of longitudinal management rather than failure of the primary strategy.

Pro00013608, approved on July 1, 2021.