Long-Term Outcomes of Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries.

Background: While complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is achieved with low surgical mortality, this condition poses ongoing mid- and late-term mortality. The long-term data for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries at a single institution are presented in this paper.

Methods: We reviewed the outcomes for 100 patients with PA/VSD/MAPCAs who had surgery between January 1987 and July 2018. Two patients were excluded who had palliative surgery not intended to reach a complete repair.

Results: Of the 98 patients who were suitable candidates for complete repair, 13% (13/98) had primary complete repair and 64% (63/98) had staged complete repair. Estimated survival at 1, 5, 10, and 20 years of age was 93%, 87%, 81%, and 76%, respectively. The median age of those patients alive at follow-up was 15 (interquartile range 9-21) years. Freedom from reintervention after staged complete repair was 58% at 5 years after complete repair. Ligating or embolizing MAPCAs was associated with a decreased hazard of death in the staged complete repair group (hazard ratio 0.6, p-value 0.04).

Conclusions: A strategy of staged complete repair can be used to achieve complete repair and stable long-term survival for patients with PA/VSD/MAPCAs.