Biventricular repair for common atrioventricular canal defect with parachute left atrioventricular valve

Objectives: Parachute left atrioventricular (AV) valve can complicate repair of common atrioventricular canal (CAVC), and single-ventricle palliation is sometimes preferred. The goal of this study is to review our single institutional experience in biventricular repair in this patient group.

Methods: The demographic, procedural and outcome data were obtained for all children who underwent biventricular repair for complete CAVC with parachute [single left ventricular (LV) papillary muscle] or forme fruste parachute left AV valve (closely spaced LV papillary muscles) from 2001 to 2012. Primary outcomes were survival, freedom from left AV valve stenosis (defined as an inflow gradient ≥7 mmHg and post-capillary pulmonary hypertension) and freedom from left AV valve replacement.

Results: A total of 24 patients were included (21 parachutes, 3 forme frustes). There was 1 early death (4.2%). At discharge, no patient had more-than-mild regurgitation and 1 had stenosis. During a median follow-up of 3.7 years (IQR 4 months to 5 years), there were 2 late deaths (8.3%), 6 patients (25%) presented significant left AV valve stenosis and 2 patients (8.3%) required valve replacement. Freedom from stenosis was 95 ± 4.9% at 1 year, 83.1 ± 8.9% at 3 years, 64.7 ± 13.5% at 5 years and 51.7 ± 15.8% at 10 years. Complete cleft closure was not associated with a significantly different freedom from left AV valve reoperation (log-rank test, P = 0.89) or significant stenosis (P = 0.47).

Conclusion: Biventricular repair in parachute left AV valve and CAVC is feasible with acceptable mortality and freedom from stenosis. The burden of reoperation remains significant in this patient group.