Staged correction of pulmonary atresia, ventricular septal defect, and collateral arteries

Objectives: Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCAs) presents with variable anatomy with regard to the pulmonary vasculature, requiring personalized surgical treatment. A protocol consisting of staged unifocalization and correction was employed.

Methods: Since 1989, 39 consecutive patients were included (median age at first operation 13 months). In selected cases, a central aorto-pulmonary shunt was performed as the first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure, and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Echocardiographic data were obtained postoperatively and at interval follow-up.

Results: In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, of which four have since died. One patient is awaiting further correction. A correction was performed successfully in 28 patients. Operative mortality was 3% and late mortality was 11%. Median follow-up after the correction was 19 years. Eleven patients required homograft replacement. Freedom from conduit replacement was 88%, 73%, and 60% at 5, 10, and 15 years respectively. Right ventricular function was reasonable or good in 75% of patients. All but one patient were in NYHA Class I or II.

Conclusions: After complete unifocalization 30/37 patients (81%) were considered correctable. The staged approach of PA, VSD, and SPCAs results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.