Background: Congenital heart defects (CHD) are the most common birth defect and are a major cause of childhood illness and death. Recent progress in management of persons with CHD may have decreased CHD-related mortality.
Methods: Year 2000 US death records were used to determine CHD-related mortality by age, sex, and race/ethnicity in children and adults. CHD-related mortality was defined as all deaths with any mention of CHD on the death certificate. Age-, sex-, and racial/ethnic-specific population counts were obtained from the 2000 US Census and used as denominators in mortality rates.
Results: In 2000 there were 5441 (.23%) CHD-related deaths and CHDs were mentioned 6121 times as the underlying or contributing cause of death. In 68.4% of CHD-related deaths, CHD was the underlying cause of death. Non-Hispanic Black males had greater risk of CHD-related death than did non-Hispanic White males (RR 1.25, 95% CI 1.08-1.45). Both Hispanic males and females had lower rates of CHD-related deaths than did non-Hispanic Whites (RR .72, 95% CI .60-.85; RR .52, 95% CI .42-.65, respectively). "Unspecified congenital malformation of the heart" was the most common cause of death overall; however, "malformation of the coronary vessels" was most often a cause of death for non-Hispanic Blacks and children aged 10-19 years.
Conclusions: Racial/ethnic differences in CHD-related mortality exist in the United States. Management of CHD, access to adequate care, and misclassification in cause of death reporting on death records may explain the observed differences.