Staged repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 104 patients

Objective: To determine the early and intermediate-term outcome of the staged repair used to treat children with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Methods: We reviewed a retrospective case series of 104 patients with this complex lesion. Information was obtained from medical records and referring physicians. Results: Of the 104 patients treated with the staged repair, 58 achieved completion of anatomic repair. The 10-year mortality was 16.5%. In the patients with complete repair, the median right-to-left ventricle pressure ratio was 0.5. The overall surgical reoperation rate was 17%, and 15.5% of patients required postoperative interventional cardiac catheterization. In the multivariate analysis, the number of collateral vessels incorporated in the repair was found to be an independent risk factor for postoperative mortality and an elevated right-to-left ventricle pressure ratio after complete repair. Conclusion: The staged repair can be successfully used to treat patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. This method yields a relatively low mortality with good functional results.