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DOI: 10.1055/s-2007-967614
18 years of experience with Fontan palliation – „about mistakes we made in the early days“
Aims: Retrospectively, we report on our longterm results of Fontan palliation to demonstrate the influence of various strategies in „Total Cavopulmonary Connection“ (TCPC) for palliative therapy of patients with „single ventricle“ physiology.
Methods: From 1988 to 2006, a total of 74 patients (mean age 4.8±3.6 years) underwent definitive TCPC at our unit. 66% of our patients underwent one-stage TCPC, in 34% we performed a two-stage modified Fontan operation; 28 patients had central fenestration (4mm), our last 19 patients were palliated with extracardiac Fontan method. Inhalative NO therapy in the immediate postoperative period was adopted in 1993.
Results: Overall 5-year survival was 85.4%, after two-stage TCPC 87.5%, and 86.3% in patients undergoing fenestrated procedures. Two of three patients survived perioperative Fontan take-down. We lost 11 patients (nine early and two late deaths): three patients died primarily because of neurologic dysfunction and eight patients because of cardiac failures. In extracardiac Fontan patients mortality was 0%. Under perioperative NO therapy there was no early death. After a mean follow-up of 67.9±23.3 months, 81% of all patients were in NYHA I and 19% in NYHA I-II. 89.7% had sinus rhythm. 29% of our patients suffered from temporary pleuropericardial effusions.
Conclusions: Definitive palliation with TCPC achieves acceptable clinical results. Two-stage repair, fenestration, extracardiac Fontan approach and postoperative inhalative NO therapy – each have a positive influence on early and long-term survival.