Atrioventricular septal defect epidemiology and demographics

Epidemiology and demographics
In the US, the prevalence of atrioventricular septal defects is between 3-5% in live births or about 0.19 per 1000 live births. The incidence of AVSD is higher in stillborn births, at a rate of approximately 7%. One explanation for this increase is this population's propensity for chromosal and other anomalies. Of live births, approximately 45% of infants with Down syndrome and congenital heart disease have an atrioventricular canal defect. Atrioventricular septal defects are distributed about equal between males and females.

There is some evidence to suggest a potential link between atrioventricular septal defects and familial clustering. Approximately 14% of women with a common atrioventricular canal pass on the congenital heart disease to their children. In one analysis, 11.7% of children with atrioventricular septal defects had a family history of congenital heart disease

There is no epidemiological evidence to support any variation of incidence and prevalence based on race, sex or age. Onset is generally early in life, occurring within the first 6 weeks of life. In rare cases, patients can be asymptomatic during development and may experience a latent onset as cyanosis develops into a severe complication.