Impact of Improved Survival in Congenital Heart Disease on Incidence of Disease

Abstract

Survival rates and life expectancies for patients with congenital heart disease (CHD) have dramatically increased, and these patients are now reaching reproductive age. As they reproduce, questions pertaining to recurrent risk of disease and the impact on incidence rates have emerged. Recurrence rates for CHD have been estimated at 3% to 5%, although, due to the complex genetics underlying CHD, this range may represent an underestimation of the true risk. Debate still exists on whether the impact of recurrence of disease has been reflected in incidence rates. Although incidence rates have undoubtedly increased, the mechanism underlying this remains unclear; improved detection likely accounts for the majority of the observed increase; however, a true increase may be present simultaneously. Concurrently, certain factors, including improved fetal detection and elective terminations, infertility, increased rates of spontaneous abortion and intrauterine fetal demise in women with CHD, and folic acid supplementation, are leading to decreases in the incidence of CHD. It is likely that the full impact of improved survival and heritability of CHD on incidence rates remains to be seen and will likely be attenuated by other factors acting to decrease incidence.