[Repair of complete atrioventricular canal before one year of age].

We report a series of 22 children with complete atrioventricular canal (CAVC) operated upon before the age of one year. The youngest patient was 1 month old and weighed 3 kg. The patients' mean age was 7 months and their mean weight was 5.4 kg. 15 patients had trisomy 21, and in one patient the CAVC was associated with tetralogy of Fallot. The remaining 21 patients had congestive heart failure resistant to medical treatment, with clinical evidence of pulmonary arterial hypertension (PAHT). At the time of surgery, 2 patients had been under artificial respiration for one month. All patients were explored by echocardiography and cardiac catheterization. The mean pulmonary pressure/aortic pressure ratio was 0.92; the mean pulmonary flow rate/systemic flow rate ratio (Qp/Qs) was 2.9/1 and the mean pulmonary resistance/systemic resistance ratio (Rp/Rs) was 0.22. All children were operated upon under deep hypothermia with circulatory arrest (mean 54 min); the patient with tetralogy of Fallot had an additional period of extracorporeal circulation. Fourteen patients had Rastelli's type A CAVC and 8 had type C CAVC. All were operated upon by the classical Rastelli technique, using a single autologous pericardial patch; in none of the patients was the septal "slit" or "commissure" entirely closed. Three patients died within 48 hours of the operation: the first one died of sudden low cardiac output 18 hours after surgery, the second one of persistent PAHT and the third one of malignant hyperthermia. The patient under artificial respiration before surgery could not be disconnected and died on the 30th post-operative day.(ABSTRACT TRUNCATED AT 250 WORDS)