The influence of different strategies on clinical outcome in patients undergoing total cavopulmonary connection

Background: We report on results of a prospective clinical trial designed t o demonstrate the influence of various strategies in "Total Cavopulmonary C onnection" (TCPC) for palliative therapy of patients with "single ventricle " physiology. Methods: From 1989 to 1997, a total of 47 patients (mean age 4.8 +/- 3.6 years) underwent definitive TCPC at our unit. 31 patients (66%) underwent one-stage TCPC, in 16 patients (34%) we performed a two-stage mo dified Fontan operation; 21 patients had central fenestration (4 mm). Inhal ative NO therapy in the immediate postoperative period was adopted in 1993. Results: Overall 5-year survival was 76.4%, after two-stage TCPC 87.5%, an d 81.3% in patients undergoing fenestrated procedures. Two of three patient s survived perioperative Fontan take-down. We lost 11 patients (nine early and two late deaths): three patients died primarily because of neurologic d ysfunction and eight patients because of cardiac failures. Under perioperat ive NO therapy there was no early death. After a mean follow-up of 35.9 +/- 23.3 months, 76% of all patients were in NYHA I and 21% in NYHA I-II. 89.7 % had sinus rhythm. 42% of our patients suffered from temporary pleuroperic ardial effusions. Conclusions: Definitive palliation with TCPC achieves acc eptable clinical results. Two-stage repair, fenestration, and postoperative inhalative NO therapy - each have a positive influence on early and long-t erm survival.