Mortality associated with adult congenital heart disease: Trends in the US population from 1979 to 2005

BackgroundSignificant advances over the last 5 decades have allowed most patients with congenital heart disease to survive well past childhood and into adulthood. Population-based data from the United States are limited regarding mortality in adult survivors.MethodsWe used the Center for Disease Control Multiple Cause-of-Death registry to determine trends in mortality from 1979 to 2005 among individuals with congenital heart disease in the United States.ResultsThere were significant reductions in death rates for adults with a number of congenital defects including ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, and Ebstein anomaly. Notably, when all ages were analyzed, there was a 71% decline in deaths associated with transposition of the great arteries (P = .001) and a 40% reduction in deaths associated with tetralogy of Fallot (P < .001). Mortality related to other lesions declined as well. Among adults with cyanotic lesions, the primary contributing cause of death was arrhythmia followed by heart failure. For adults with noncyanotic lesions, the major contributing cause before 1990 was arrhythmia; after 1990, myocardial infarction became the leading contributing cause of death. There was an overall decrease in the incidence of arrhythmia as the cause of death in all ages, particularly among children.ConclusionsPatients with congenital heart disease are living longer. Arrhythmia remains the primary contributing cause of death for those with cyanotic lesions. Myocardial infarction is now the leading contributing cause for adults with noncyanotic congenital heart disease consistent with late survival and an increasing impact of acquired heart disease.