Improving Results of the Fontan Procedure in Patients With Heterotaxy Syndrome

Background The Fontan operation in patients with heterotaxy syndrome has been associated with a poor prognosis. We studied whether the outcome of those patients with heterotaxy syndrome improved compared with those who did not have the syndrome after an extracardiac Fontan operation. Methods A retrospective review was performed on 185 patients who had undergone an extracardiac Fontan operation between 1996 and 2005. Results Sixty-two of the patients had heterotaxy syndrome (33.5%). Forty-one had right isomerism and 21 had left isomerism. Heterotaxy syndrome was commonly associated with a morphologic right ventricle (59.7%), a common atrioventricular valve (72.6%), an interrupted inferior vena cava (25.8%), a separate hepatic vein (30.6%), and extracardiac pulmonary venous drainage (16.1%). The hospital mortality rate was higher in the heterotaxy syndrome than nonheterotaxy (4.8% vs 2.4%; p = 0.05). Eight-year survivals were 91.9 ± 3.2% in the nonheterotaxy group and 89.3 ± 4.2 % in the heterotaxy group (p = 0.39). At 8 years, freedom from reoperation was 90.2 ± 3.2% in the nonheterotaxy group and 78.5 ± 6.2% in the heterotaxy group (p = 0.15). The outcomes (other than those of arrhythmia) were no different between the two groups. The incidences of early and late postoperative arrhythmia were 29.0% and 25.4%, respectively, in heterotaxy patients, and 15.4% and 10.8% in nonheterotaxy patients (p < 0.05). Bradyarrhythmia was found to be more common. During follow-up, atrioventricular valve regurgitation of more than mild was more common in heterotaxy patients (33.9% vs 18.9%; p = 0.05). Conclusions Midterm outcomes after an extracardiac Fontan operation in heterotaxy and nonheterotaxy patients are similar, except arrhythmia and atrioventricular valve regurgitation.