DERIVATIONS CAVO-PULMONAIRES TOTALES. RESULTATS A COURT TERME

In order to reduce the complications of the Fontan procedure, 2 techniques of total cavopulmonary connection using a right intraatrial baffle (Pugas' technique, 19 patients), and a modified de Kreutzer (19 patients) were assessed in 38 consecutive patients operated between November 1980 and November 1991. All patients had complex cyanotic congenital heart disease: tricuspid atresia (13), single ventricle (12), hypoplasia of one of the two ventricles (8), pulmonary atresia with an intact septum (5). The average age was 5.8 ± 5.6 years (range 1 to 30 years), the weight was 18 ± 11.7 kg (range 8 to 59.7 kg). Six patients (15.8%) died, 4 of whom (10.5%) in the operative period. Five of these deaths were related to extremely high right heart pressures without any technical fault, and the other to thrombosis of the conduit in a patient not taking anticoagulants. Two immediate and 10 secondary reoperations were necessary. All 3 patients who had disconnection of the anastomosis because of high right heart pressures died. The postoperative course was often difficult: right heart failure (63%), pleural effusion (57%), and pericardium effusion (13%). Of the risk factors analysed, age had no influence on postoperative outcome. The two poor prognostic factors were: presence of atresia or hypoplasia of the systemic atrioventricular valve (mortality 42.8% versus 6.5%, p = 0.05 and prolongation of the hospital period: 39.5 ± 32 days versus 21 ± 11 days, p < 0.05) and previous banding (reoperation rate 62.5% versus 16.6%, p < 0.05, and longer stay in the intensive care unit and hospital period). With an average follow-up of 16.2 ± 10.5 months (range 1 to 36 months) the qualitative results are very good, all patients being in Stage I of the NYHA Classification except for one in Stage II, without any secondary arrhythmias. Total cavopulmonary connection offers a curative perspective for a large number of patients with complex cyanotic congenital heart disease. It requires very strict preoperative evaluation of pulmonary vascular resistances and very careful management in the postoperative period.