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

Background

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.

Methods

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.

Results

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 < 0.001). A longer mean event-free survival time was found in patients with normal, well-developed NPAs (p = 0.047). Finally, patients with poorly developed or absent NPAs had worse survival rates when a surgical approach was followed. Systemic-pulmonary shunt placement or unifocalisation had limited effect on prognosis in the absence of total repair (p = 0.167).

Conclusions

Patients with PA-VSD-MAPCAs who underwent complete intracardiac repair and/or with well-developed native pulmonary arteries had the best prognosis. Our analyzed data suggest that incomplete surgical repair resulted in survival rates comparable to those seen with a non-surgical approach.