Epidemiology, definition, and classification of adult congenital heart disease

Abstract

Congenital heart disease is defined as an abnormality in the cardiocirculatory structures or function that is present at birth time, even if it is revealed much later. Birth prevalence of congenital heart disease (CHD) is overall presumed to be about 0.8 %, although this does not take into consideration regional and local differences and the two most common cardiac abnormalities: congenital bicuspid aortic valve and also prolapse of the mitral valve. There is a variation in the incidence rate of CHD between 1.2 and 17 per 1,000. In the United States, now, there are more patients over 20 years old with CHD rather than under that age. Given new surgical mortality rates of less than 5 %, one would imagine that in the next decade, nearly 1 in 150 young adults will have some degree of CHD. Indeed, there are a considerable number of young adults with single-ventricle physiology, systemic right ventricles, or complex intracardiac baffles who are now entering adult life cycle and starting families. Overall mortality rate of CHD decreased by 40 % between 1979 and 1997. However, the annual hospitalization rate in adults with CHD has more than doubled in the United States between 1998 and 2005. Also during this period of time, hospitalization for adults with complex CHD increased up to 60 %. Sex differences in the incidence of CHD at birth are very well recognized previously. Atrial septal defect, mitral valve prolapse, Ebstein’s anomaly, patent ductus arteriosus, and common atrium show a strong female dominance, whereas aortic valve stenosis, aortic coarctation, transposition of the great arteries, pulmonary and tricuspid atresia, and tetralogy of Fallot occur more frequently in males. Interestingly in Asia, comparatively more right-sided and less left-sided lesions have been described in previous reports. Based on the need for the delivery of appropriate healthcare and pattern of visit, ACHD patients have been categorized as simple, moderate, and great complexity. Adult patients with simple CHD can frequently be attended to in the general medical and health community. Adults with CHD of moderate complexity must be seen regularly at adult CHD centers, and patients with great complexity must be seen periodically at local adult CHD centers.