[Long-term results of atriopulmonary fontan connection for complex cardiac anomalies]

To evaluate the long-term results of atriopulmonary Fontan connection, we reviewed the outcome of 20 consecutive patients (single ventricle 8, double outlet right ventricle 8, tricuspid atresia 3, mitral atresia 1; mean age, 13 +/- 11) who underwent this operation between 1981 and 1997 at our institution. Glenn shunt preceded Fontan operation in 1 patient. Four patients had a concomitant bidirectional Glenn anastomosis at the time of the Fontan operation. Follow-up was 100% complete, and total cumulative follow-up of all patients was 241 patient-years (maximum, 20 years). Three hospital deaths and 5 late deaths were observed, resulting in 58 +/- 11% of cumulative survival at 20 years after the operation. Sudden death probably due to arrhythmias was the major cause of the late death, and occurred in 3 patients. Reoperation was required in 5 patients (2%/patient-year) at 1 to 17 years postoperatively. Re-closure of an atrioventricular valve was performed for failure of the valve closure in 3 patients at 1 to 6 years postoperatively. Reconstruction of the stenotic atriopulmonary connection (APC) was concomitantly required in 2 of the 3 patients. Pacemaker implantation was required for bradycardia with atrial fibrillation in 1 patient at 17 years. One patient with atrial flutter developed intraright atrial thrombosis at 12 years after the initial Fontan operation, and this patient underwent conversion to extracardiac conduit cavopulmonary connection with right atrial maze procedure. Atrial tachyarrhythmias are most common complications, and occurred in 12 patients (5.0%/patient-year), resulting in 40 +/- 13% of the cumulative free rate at 15 years after the operation. Thus, failure of the atrioventricular valve closure and stenosis of APC were important early to intermediate complications after the atriopulmonary Fontan connection. On the other hand, atrial tachyarrhythmias were inevitable, and were most common late complications leading to intra-right atrial thrombosis, which was successfully resolved by conversion of the conventional atriopulmonary Fontan anastomosis to extracardiac conduit cavopulmonary connection.