Outcomes of Patients with Pulmonary Atresia and Major Aortopulmonary Collaterals Without Intervention in Infancy.

Treatment of pulmonary atresia with major aortopulmonary collaterals (PA MAPCAs) remains a challenge. Despite variations in surgical technique, contemporary strategies all include initial intervention in the first year of life. However, a subset of patients presents later in life, and contemporary outcomes of this group have not been reported previously. We performed a retrospective case series of consecutive cases of PA MAPCAs who were seen at our center between January 2001 and February 2016, who had not undergone surgery before the age of 1 year. We describe their presenting characteristics, operative and transcatheter interventions, and outcomes. A total of eight cases were identified from 76 children with PA MAPCAs treated over the study period. Median age at presentation was 5.9 years. Seventy-five percent had confluent pulmonary arteries with a median Nakata index of 113 mm2/m2. Operative intervention was performed in 5/6 cases. Two are awaiting intervention. The combination of operative and transcatheter interventions allowed for ventricular septal defect closure in 60 % of cases, all of whom had subsystemic right ventricular pressures. Operative intervention is possible in some older cases with PA and MAPCAs. Though multiple operations and transcatheter therapies are necessary, some can achieve operative correction of serial circulation with tolerable physiology. Subjects with ventricular hypoplasia and those without confluent pulmonary arteries are more challenging.