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Abstract
Significant 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.
We 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.
There 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.
Patients 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.