Repair of Atrioventricular Septal Defect Associated With Tetralogy of Fallot or Double-Outlet Right Ventricle: 30 Years of Experience

Abstract

The surgical outcomes of atrioventricular septal defect (AVSD) associated with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV) have improved in recent times. However, high mortality and reoperation rates are still reported. This study reviews our surgical experience in patients with complete AVSD and TOF or DORV. Between 1980 and 2010, 48 consecutive patients with AVSD associated with TOF (n = 26) or DORV (n = 22) underwent complete repair; of which, 19 had staged repair. A transatrial-transpulmonary approach with the 2-patch technique was the preferred surgical technique. Data were obtained from inpatient and outpatient medical files. The mortality rates were 8.3% (4 of 48) for in-hospital death and 13.6% (6 of 44) for late death. Mean follow-up was 8.0 ± 8.7 years (median 10.8 years; range from 2 months to 30 years). Actuarial survival was 76% at 5 years and 71% at 20 years. No deaths occurred after 2001. Reoperations were required in 16 of 48 patients (33%). The overall freedom from reoperation was 55% at 5 and 20 years. Down syndrome was protective for reoperation (p = 0.022). Complete AVSD associated with TOF or DORV can be repaired with good survival. Detachment of the superior bridging leaflet provided excellent exposure. However, reoperation rate remains high.