Atrioventricular Septal Defect with Aortic Arch Obstruction: 17-Years of Single Center Experience

Background: Optimal surgical treatment for atrioventricular septal defect (AVSD) with obstruction of the aortic arch including aortic coarctation (CoA) is still not standardized. Individually tailored management is required to optimize results.

Methods: The outcome of patients operated at our center from 1995 to 2012 was studied in retrospective cohort. Primary repair and staged approach for the correction of all lesions were compared.

Results: Among 459 biventricular repairs of AVSD in the studied interval, 28 (6%) had aortic arch obstruction. Primary repair was undertaken in 10 patients and 18 were staged with two surgical steps. The median age at first operation was 39.5 days (5 – 825). In both groups three patients died (0 – 105 d postoperatively). The median follow up was 48 months (3 – 198) with a survival of 83% after 2.9 months after correction. Freedom from mitral regurgitation more than moderate and mitral valve reoperation after 65 months were 76 and 78%, respectively. Aortic arch obstruction occurred only in the first 7 months after operation. Left ventricular outflow tract obstruction (LVOTO) remained a major problem with freedom from LVOTO resection of 64% after 97 months.

Comparing the two groups (1 vs. 2 stage and neonate vs. older), there was no significant difference in outcome with no late mortality of the initial survivors. All patients are asymptomatic.

Conclusion: Early mortality and morbidity of this challenging group of patients remains an issue for both primary and staged approach. Primary repair in neonates should be reserved for critically ill patients. Mid-term outcome, however, seems to be acceptable.