Objective: The Fontan procedure is well established in the treatment of single-ventricle congenital heart disease. Circulatory failure can occur acutely in the post-operative period or as a late complication, and often necessitates cardiac transplantation. The surgical risk in this patient group has been assumed to be unusually high.

Methods: We retrospectively assessed outcomes in patients that underwent transplantation for the failing Fontan circulation in a regional cardiopulmonary transplant referral centre.

Results: Twenty-seven patients underwent cardiac transplantation after either acute or delayed failure of the Fontan Circulation. In 8 patients, acute failure of the Fontan circulation necessitated urgent listing and transplantation. Twenty-one (78%) of those undergoing transplantation for this indication were under the age of sixteen years. Patients with left systemic ventricular morphology had a longer surgical palliation before transplantation (median 11.8 vs. 8.1 years for right ventricular morphology, p<0.05). Similarly, those born earlier during the study period underwent transplantation at a greater age (median 14.3 vs. 7.4 years for those born before and after 1990 respectively, p<0.001). Despite a higher early post-operative mortality risk, survival in the paediatric post-Fontan group has been similar to that in paediatric patients undergoing transplantation for cardiomyopathy (Figure 1).

Conclusions: Cardiac transplantation is a viable treatment option for patients with a failing Fontan circulation. In a single centre, survival post transplantation has been similar to survival after transplantation for cardiomyopathy in the same age group.