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Background Atrioventricular septal defect (AVSD) is a cardiac malformation that accounts for up to 5% of total congenital heart disease, occurring with high frequency in people with Down Syndrome (DS). We aimed to establish the surgical challenges and outcome of medical care in different types of AVSD in children with DS compared to those without DS (WDS). Methods The study included 62 children (31 with DS) with AVSD, evaluated over a 5 year period. Results Complete AVSD was observed in 49 (79%) children (27 with DS). Six children ha