Mid-term follow-up after extracardiac Fontan operation

Objectives: We analyzed the mid-term results after extracardiac Fontan operation (ECFO) with respect to the incidence of new arrhythmias, anthropometric development of the children, and prosthetic conduit status. Methods: Between 11/95 and 12/02 ECFO was performed in 51 children and 9 adults. ECFO was established using Gore-Tex® conduits with a median diameter of 20 mm. Thirty patients underwent postoperative heart catheterization and angiography. Results: Total early mortality was 6.6% (four patients). There was no low cardiac output and there were no deaths in 30 children under 4 years of age. Forty-eight patients (94%) with preoperative sinus rhythm retained it postoperatively. A permanent pacemaker for bradyarrhythmia was required in one child 2 years after surgery. During the median follow-up of 5.0 years no further arrhythmias and no late deaths occurred. One re-operation for replacement of the oversized conduit was performed in a 3-year-old child with severe protein-losing enteropathy 6 months after surgery. Three patients with mild conduit compression or stenosis were treated successfully by transcatheter intervention. Children operated on before the age of 4 years showed a manifestly accelerated postoperative weight gain toward normal (p < 0.05), compared to older children. Conclusions: The mid-term results after extracardiac Fontan operation show low mortality and morbidity rates and a low incidence of postoperative arrhythmias. In the majority of patients implanted conduits remain stable without any development of stenosis or thrombosis. Accelerated somatic development, with normalization of weight especially in small children, is to be observed after completion of the Fontan circulation.