Treatment Strategies and Outcomes in Pulmonary Atresia With Ventricular Septal Defect Without Major Aortopulmonary Collateral Arteries in North America 2003-2023.
Document Type
Article
Publication Date
2-2026
Identifier
DOI: 10.1016/j.athoracsur.2025.07.044
Abstract
BACKGROUND: 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.
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 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.
RESULTS: 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.
CONCLUSIONS: Early complete repair of PA/VSD without MAPCAs is gaining prominence and yields short-term and midterm outcomes comparable to staged approaches.
Journal Title
The Annals of thoracic surgery
Volume
121
Issue
2
First Page
412
Last Page
420
MeSH Keywords
Humans; Pulmonary Atresia; Female; Male; Treatment Outcome; Retrospective Studies; Hospital Mortality; Infant; Infant, Newborn; Heart Septal Defects; North America; Pulmonary Artery; Cardiac Surgical Procedures; Collateral Circulation; Survival Rate
PubMed ID
40848887
Keywords
Pulmonary Atresia; Treatment Outcome; Retrospective Studies; Hospital Mortality; Heart Septal Defects; North America; Pulmonary Artery; Cardiac Surgical Procedures; Collateral Circulation; Survival Rate
Recommended Citation
Abdul Ghayum MA, Contorno E, Lee B, O' Brien J, Bedzra E. Treatment Strategies and Outcomes in Pulmonary Atresia With Ventricular Septal Defect Without Major Aortopulmonary Collateral Arteries in North America 2003-2023. Ann Thorac Surg. 2026;121(2):412-420. doi:10.1016/j.athoracsur.2025.07.044
COinS
