A retrospective study: Long term prognosis in adults with PA-VSD-MAPCAs

• Published in: International journal of cardiology 2024-11, Vol.415, p.132476, Article 132476
• Main Authors: Berghmans, Sophie, Eyskens, Bénédicte, Rega, Filip, Moons, Philip, Troost, Els, De Meester, Pieter, Van De Bruaene, Alexander, Budts, Werner
• Format: Article
• Language: English
• Subjects: Cardiac and Cardiovascular Systems; Kardiologi; MAPCA; Pulmonary atresia; VSD
• ISSN: 0167-5273; 1874-1754; 1874-1754
• DOI: 10.1016/j.ijcard.2024.132476

Pulmonary Atresia, Ventricular Deptal Defect, and Major Aortopulmonary Collateral Arteries (PA-VSD-MAPCAs) is a congenital cyanotic heart defect with poor prognosis. Due to its complex and highly variable anatomy, the best treatment plan is not clear. We aimed (1) to investigate the survival of PA-VSD-MAPCAs patients according to the underlying original anatomy and treatment strategy, and (2) to evaluate life expectancy between patients with or without severe hypoplastic native pulmonary arteries (NPAs) after surgical versus non-surgical treatment. A prospectively established database of 169 PA-VSD-MAPCAs patients treated and followed up at University Hospitals Leuven was accessed. Patients were divided into three groups according to the treatment strategy. Kaplan-Meier survival curves were plotted, and Log Rank tests were used for comparison. The overall mean survival for patients with PA-VSD-MAPCAs was 38.5 years (95%-CI: 33.1–43.9). Patients with complete intracardiac repair had the longest mean survival of 43.8 years (95%-CI: 38.1–49.6) versus the other groups (p