Long-term outcomes of atrioventricular septal defect and single ventricle: a multicenter study.

Abstract Objective To analyze survival and incidence of Fontan completion of single ventricle patients with concomitant unbalanced atrioventricular septal defect (AVSD). Methods Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with AVSD-single ventricle were selected: 36 patients underwent an atrioventricular valve (AVV) procedure (valve surgery group). Endpoints were survival, incidence of Fontan completion and freedom from AVV re-operation. Results Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70% and 68.5% at 10, 15 and 20 years. An AVV procedure was not a risk factor for mortality. Patients with moderate-severe or severe AVV regurgitation at echocardiographic follow-up had a significantly worse 15-years survival (58.3%) compared to patients with none or mild (89.2%) and patients with moderate regurgitation (88.6%) (p=0.033). Cumulative incidence of Fontan completion was 56.5%, 71% and 77.6% at 5, 10 and 15 years. An AVV procedure was not associated with the incidence of Fontan completion. In the valve surgery group freedom from AVV re-operation was 85.7% at 1 year and 52.6% at 5 years. Conclusions The long-term survival and incidence of Fontan completion in our study were better than previously described for single ventricle patients with AVSD. A concomitant AVV procedure did not increase the mortality rate nor decrease the incidence of Fontan completion, whilst patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single ventricle and AVSD when AVV regurgitation exceeds a moderate degree, the AVV should be repaired.