Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome

• The atrioventricular canal is the "classic" congenital heart anomaly in Down syndrome. We may learn more of the nature of this disorder by careful study of the anatomic characteristics of the cardiac lesions and by comparing these lesions in patients with and patients without Down syndrome. We reviewed the clinical characteristics (echocardiographic and angiocardiographic) of 220 patients with atrioventricular canal and compared the prevalence of anatomic types and associated cardiac malformations in children with (105) and without (115) Down syndrome. In patients with Down syndrome, the complete form of atrioventricular canal was prevalent, with a high frequency of associated Fallot's tetralogy. Partial atrioventricular canal and left-sided anomalies were more common in patients without Down syndrome. Down syndrome is associated with a simpler type of atrioventricular canal when compared with patients with a normal chromosome configuration. ( AJDC . 1990;144:1120-1122)