Background: The purpose of this study was to define the prevalence of specific sequelae after extracardiac Fontan operation.
Methods: Sixty-five consecutive patients undergoing extracardiac Fontan operation were studied for mortality, Fontan failure, systemic ventricular function, supraventricular arrhythmias, thromboembolism, and growth potential. Age was 3 to 31 years (mean +/- standard deviation, 9.4 +/- 1.8; median, 7 years). The conduits were constructed of polytetrafluoroethylene (n = 50), and "viable" in situ pericardium (n = 15). The patients underwent serial echocardiogram, dynamic radionuclide studies, and cardiac catheterization.
Results: Operative mortality was 3%, and the incidence of conduit thrombosis was 4.6%. There was paradoxic filling of the right lung after femoral injection of the radiotracer in all cases of conduit obstruction. Perioperative and late postoperative supraventricular arrhythmias were observed in 9.2% and 4.7% of patients, respectively. Risk factors for supraventricular arrhythmias included systemic ventricular dysfunction (p = 0.000), heterotaxy syndrome (p = 0.008), systemic venous anomalies (p = 0.015), and previous bidirectional Glenn operation (p = 0.017). At a mean follow-up of 77 +/- 2 months (range, 8 to 79 months), there were no late deaths (actuarial survival at 79 months, 96.9% +/- 0.02%). Serial echocardiograms demonstrated evidence of growth of the viable tunnels. Postoperatively, there was transient depression of ejection fraction in all patients (p = 0.000).
Conclusions: Supraventricular arrhythmias after extracardiac Fontan are more common in patients with heterotaxy syndrome, bilateral superior venae cavae, systemic ventricular dysfunction, and those undergoing completion Fontan operation. The viable tunnel may emerge as an optimal alternative by virtue of reduction of supraventricular arrhythmias, elimination of the need for anticoagulation, and addressing the issue of growth potential in selected patients.