Results of total correction of tetralogy of Fallot with complete atrioventricular canal.

Four patients with tetralogy of Fallot (TF) and complete atrioventricular canal (CAVC) underwent successful intracardiac repair. All patients had undergone preliminary palliative shunts. Associated cardiovascular lesions included muscular ventricular septal defect (VSD) and left superior vena cava (one patient), bilateral main pulmonary artery stenosis (one patient), and right aortic arch (two patients). The diagnosis was suspected clinically by electrocardiographic evidence of right ventricular hypertrophy and left anterior hemiblock, by echocardiographic findings suggestive of CAVC with aortic dextroposition, and by the association with trisomy 21. The diagnosis was confirmed by cardiac catheterization and cineangiography. Total correction consisted of closure of the VSD and atrial septal defect (ASD), reconstruction of the atrioventricular valves, and relief of the right ventricular outflow tract obstruction (RVOTO). There were no early or late postoperative deaths. Surgically induced complete heart block did not occur. One patient underwent successful reoperation for residual VSD and right ventricular outflow aneurysm. Late hemodynamic evaluation revealed good results in all patients. We recommend a staged treatment plan consisting of preliminary systemic--pulmonary artery shunting for symptomatic children under 4 to 5 years of age and total correction for older children. With proper preoperative diagnosis and accurate intracardiac repair, good results may be obtained after total correction of TF and CAVC.