American Family Physician

T he number of adults in North America who have congenital heart disease (CHD) has steadily increased over the past 30 years. Although this increase is partly because of the growth of the adult population, it may be more attributable to improvements in surgical management, which have resulted in a greater than 90 percent 10-year survival rate in persons with CHD. Between 1979 and 1997, mortality from CHD decreased 39 percent in the United States. In North America, at least 500,000 adults have had surgical correction of CHD, with nearly 50 percent requiring two or more surgeries, and 23 percent requiring three or more. Congenital heart lesions may become symptomatic any time from birth to adulthood. At least 10 percent of persons with CHD have lesions that are typically not diagnosed until adulthood, such as secundum atrial septal defect (ASD) and congenitally corrected transposition of the great arteries (a condition in which the aorta and pulmonary artery are transposed, as are the ventricles, resulting in congenital correction of circulation). Patients with some types of common CHD lesions often survive into adulthood (e.g., those with bicuspid aortic valve, congenital forms of mitral valve prolapse, coarctation of the aorta, ASDs, pulmonary valve stenosis, patent ductus arteriosus, or tetralogy of Fallot). Diagnostic imaging procedures for adults with suspected CHD include chest radiography, echocardiography (transthoracic and transesophageal), nuclear scintigraphy, cardiac-gated computed tomography (CT), magnetic resonance imaging (MRI), and cardiac catheterization and angiography. The evaluation of CHD often requires the accurate assessment of not only intraand extracardiac anatomy, but also hemodynamics and function. Adults with CHD have the additional clinical considerations of acquired comorbidities (e.g., hypertension, atherosclerosis, occlusive coronary artery disease, pulmonary disease, renal disease) that may complicate their medical or surgical treatment.