Atrioventricular septal defect is a complex congenital heart disease in which cardiac septation is deficient especially at atrioventricular and ventricular levels. The anatomopathologic experience at the National Institute of Cardiology "Ignacio Chávez" is described; an anatomo-embryological correlation was made to gain an insight of the pathogenesis. Seventy hearts were analyzed morphologically with the sequential segmentary system.
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