ORTHOTOPIC CARDIAC TRANSPLANTATION FOR THE FAILING FONTAN CIRCULATION

Objective: Modified Fontan procedures are now employed in several cond itions unsuitable for bi-ventricular repair. Selection criteria have b een relaxed. The procedure is palliative. Longterm outlook is unknown. This study evaluated factors associated with the development of a fai ling Fontan circulation and transplantation results. Methods: Retrospe ctive review of patients referred to a single centre for cardiac trans plant assessment. Results: Between 1985 and 1996, 46 of 448 cardiac tr ansplants were performed for congenital heart disease. Nine of these w ere performed in patients with a failing Fontan circulation (four adul ts, five children). In six cases, the dominant ventricle had left vent ricular (LV) morphology. Congenital anomalies included double outlet r ight ventricle (three cases), double inlet left ventricle (two cases), tricuspid atresia (two cases), and pulmonary atresia with intact vent ricular septum (one case). Fontan procedures were performed in absence of sinus rhythm (four cases), atrio-ventricular (AV) valve regurgitat ion (two cases), aortic regurgitation and systolic LV dysfunction (one case), elevated mean pulmonary artery pressure (one case), and older age (>7 years, eight cases). Three patients required early reoperation and two needed permanent pacing. Subsequent deterioration associated with loss of sinus rhythm (four cases) and progressive AV valve regurg itation (seven cases) led to transplant assessment (at <1 year, five c ases; at 2-12 years, four cases). All patients were listed for transpl antation. Three patients required intravenous inotropic support and th ree patients with lymphocytotoxic antibodies needed prospective crossm atching. Donor cardiectomy was modified to facilitate implantation. Th e recipient operation involved pulmonary artery reconstruction (using pericardium), modified atrial and direct caval anastomoses. Three pati ents died within 24 h of surgery (two graft failures, one haemorrhage) . In operative survivors (n = 6), intensive care stay was 3-16 days, a nd hospital stay ranged from 14 to 32 days. There have been no subsequ ent deaths (follow up, 0.5-4.7 years). Conclusion: In high-risk Fontan candidates, transplantation may be preferable at the outset. Previous surgery, lymphocytotoxic antibodies, indeterminate pulmonary vascular resistance, emergency status, suboptimal donor selection, and periope rative bleeding contribute to peri-operative mortality. In survivors, the outcome remains very encouraging. (C) 1998 Elsevier Science B.V. A ll rights reserved.