Primary complete repair of interrupted aortic arch with associated lesions in infants.

OBJECTIVE: Interrupted aortic arch (IAA) is a complicated congenital heart disease requiring an individualized management strategy. We reported the results for surgical repair of IAA with associated anomalies.

METHODS: This was a retrospective review of 119 patients undergoing one-stage biventricular repair of IAA with associated lesions at the median age of 18 days (range, 3 to 90) between 2000 and 2013. End-to-side anastomosis with patch augmentation was adopted in all patients. Left ventricular outflow tract obstruction (LVOTO) procedure was performed in 23 patients. Selective cerebral perfusion was used in 55 patients (46%).

RESULTS: IAA types were A in 92 patients (77%) and B in 27 (23%). Associated anomalies were multiple including noncomplex lesions in 91 (76%) and complex lesions in 28 (24%). Mean follow-up was 98.7 ± 74.2 months. Follow-up was 80% completed. There were 19 in-hospital and six late deaths. The overall actuarial survival including early mortality was 84% at 30 day, 81% at five years, and 79% at 10 and 13 years. Cox proportional hazard model was used to determine risk factors for death: presence of complex lesions (p = 0.005), critical aortic valve stenosis (AVS) (p = 0.016), and long cardiopulmonary bypass (CPB) duration (p = 0.036). Eighteen patients required re-intervention, including 16 for subsequent LVOTO and two for arch restenosis.

CONCLUSIONS: Single-stage repair using end-to-side anastomosis with patch augmentation is an effective approach for infants with IAA.