Successful repair of double-outlet right ventricle by a modified Fontan operation

Patients having a double-outlet right ventricle with an unfavorable anatomy for reparative conduit procedure present a formidable surgical challenge. Three such patients, aged 10, 13, and 14 years, with double-outlet right ventricle associated with atrioventricular discordance in 2 and atrioventricular concordance in 1, levotransposition of the great vessels and dextrocardia. A large-diameter direct anastomosis of the right atrium to the pulmonary artery was performed after the closure of the atrioventricular and pulmonary valves. All patients were operated on successfully with good clinical and hemodynamic results. Postoperatively, all 3 were acyanotic and had an improved physical activity compared with their preoperative status. Mean cardiac index at rest was 3.23 L/min/m2 and was increased to 5.04 L/min/m2 during exercise. These results indicate that this procedure can be of value for patients with double-outlet right ventricle in whom reparative surgery is technically difficult or infeasible.