[The atrioventricular canal and vascular ring. The surgical treatment of a rare anatomical association].

We report the clinical and surgical data of 11 patients with atrioventricular canal and vascular ring. Down syndrome was present in 8 cases (72.7%); 8 patients were female (72.7%). Seven patients underwent, at the same time, correction of vascular ring and banding of the pulmonary artery with 4 death. Two children underwent correction of vascular ring and subsequent correction of atrioventricular canal without mortality. Two patients underwent correction of atrioventricular canal and subsequent correction of the vascular ring with one death. The mortality of the 6 patients operated after the age of 6 months was in measure of 16.6%. This association of malformations may be due to anomalies of neural crest and, although rare, should be particularly kept in mind referring to female patients with Down syndrome.