Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience

Abstract

Objective: The surgical results for the repair of interrupted aortic arch (IAA) have evolved in recent years. We report our results for staged repair of this complex congenital malformation. Methods: Sixty-five patients (mean age, 16.9 ± 41.7 days) were diagnosed with IAA and referred for surgical therapy. The surgical management strategy at our institution between 1982 and 2005 has been one-stage complete repair (n = 13) or staged repair (n = 52) in selected patients. Non-complex patients (group I, n = 51) had a ventricular septal defect (87%), aortopulmonary window (8%), and left ventricular outflow tract obstruction (27%). Group II (n = 14) were patients with Taussig–Bing double outlet right ventricle (n = 6) or truncus arteriosus (n = 8). Method of staged repair of IAA was to transect and turn down the left carotid artery and anastomosis it to the descending aorta (n = 41) or graft interposition (n = 2) combined with a pulmonary artery (PA) banding followed in a few months by delayed ventricular septal defect (VSD) closure and PA de-banding. Results: There were 5 early and 10 late deaths. The actuarial survival including early mortality was 92% at 1 year, 81% at 5 years, and 76% at 10 and 15 years. There was an 81% 15-year survival for children in group I compared with a 54% for children in group II (p ≪ 0.001). Risk factors for increased mortality by univariate analysis were as follows: (1) primary aortic anastomosis (p = 0.03), (2) presence of complex anomalies (p = 0.05), and (3) initial IAA repair performed before 1994 (p = 0.05). Actuarial freedom from any type of aortic reoperation or intervention was 86% at 1 year, 69% at 5 years, and 60% at 10 and 15 years. Univariate and multivariate analyses identified no tested variables as risk factors for reoperation. The majority (86%) was in New York Heart Association (NYHA) class I, and 14% remained in NYHA class II. During the postoperative course there were no neurologic deficits, seizures, and growth disturbances in any patient. Conclusion: Staged repair of IAA using a left carotid artery turn down can be safely applied in IAA patients with and without other intracardiac anomalies with good results. Use of the left carotid artery for arch reconstruction did not result in any detectable neurological events or growth disturbances later in life. Associated anomalies played an important role in outcomes. The long-term probability for reoperation and/or reintervention remains high regardless of operative technique.