PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM - RESULTS OF THE FONTAN PROCEDURE

Background. Children with pulmonary atresia and an intact ventricular septum show a heterogeneous spectrum of cardiac anomalies. A biventric ular repair is attainable in some; a Fontan procedure or a one-and-a-h alf ventricle is the only possible repair for others. Children with ri ght ventricle-to-coronary artery connections, with or without right ve ntricle-dependent coronary artery blood flow, are a high-risk group. M ethods. Between May 1980 and December 1994, 22 children underwent a Fo ntan operation for the treatment of pulmonary atresia with an intact v entricular septum at The Hospital for Sick Children, Toronto. The mean age was 5.8 years (median, 4.9 years). All children had had at least one pre-Fontan palliative procedure; 19 had two, and 7 of these had th ree or more. Right ventricle-to-coronary artery connections were prese nt in 15 children, including 5 with right ventricle-dependent coronary artery blood now. Thromboexclusion of the right ventricle was done in 10 children, with 7 undergoing it before and 3 at the time of the Fon tan procedure. Results. There were three early deaths (13.6%) and one late death. The actuarial survival at 10 years after the Fontan operat ion was 80%. Early postoperative complications occurred in 4 children. Follow-up was completed in all children at a mean of 4 years (range, 1 to 12.5 years) after the Fontan operation. Atrial arrhythmia occurre d in 3 children, and permanent pacemakers were required in 4. Conclusi ons. Results of the Fontan operation for the treatment of pulmonary at resia with an intact ventricular septum are satisfactory. Thromboexclu sion of the right ventricle is indicated in the presence of right vent ricle-to-coronary artery connections without right ventricle-dependent coronary artery blood now. The right ventricle should not be decompre ssed or thromboexcluded in children with right ventricle-dependent cor onary artery blood now, and at the Fontan operation, saturated blood m ust enter the right ventricle. (C) 1997 by The Society of Thoracic Sur geons.