Congenital Heart Disease: Incidence in the First Year of Life

The Heritage Pediatric Cardiology Program is a regional study of patients with congenital heart disease in northern and central Alberta, Canada. Cases of congenital heart disease were confirmed by clinical examination by a pediatric cardiologist plus echocardiography (noninvasive criteria) with or without cardiac catheterization, cardiac surgery, or autopsy (invasive criteria). From 1981 to 1984 inclusive, 573 confirmed cases of congenital heart disease occurred in a population of 103,411 livebirths (5.54 and 3.36/1,000 livebirths using the noninvasive and the invasive criteria, respectively). This is significantly higher (p less than 0.0001) than the rates found in the Baltimore-Washington Infant Study (3.69 and 2.38/1,000 livebirths for the noninvasive and invasive methods, respectively) or the New England Regional Infant Cardiac Program (2.20/1,000 livebirths using invasive methods). Although some lesions occur at rates similar to those found in these other registries, there are 4.4 times more double outlet right ventricles and 2.9 times more atrial septal defects in northern and central Alberta than were found in the New England Regional Infant Cardiac Program (p less than 0.001). The overall congenital heart disease rate has increased 47% between 1981 and 1984, and the rate of ventricular septal defects has doubled during the same period in northern and central Alberta. No methodological bias was found to explain these higher and increasing rates.