Lesion-specific mortality due to congenital heart disease in U.S. adults from 1999 to 2017.

Background: Adults with congenital heart disease (ACHD) comprise an increasing proportion of individuals living with CHD in the United States (US) although little is known regarding lesion or age-specific mortality in the modern era. Our study aimed to describe current era ACHD mortality rates by age and CHD lesion and provide temporal mortality rate trends for the last two decades.

Methods: We conducted a 19-year analysis (1999-2017) of publicly available, de-identified Multiple Cause of Death data compiled and produced by the National Center for Health Statistics. Age and lesion-specific mortality rates were calculated using joinpoint regression.

Results: ACHD mortality rates decreased by an average of 2%-4% per year for all adults. CHD lesions resulting in the highest ACHD-related mortality varied by age. Unlike the other lesions, mortality attributed to single ventricle physiology failed to improve in early adulthood (average of 1.6% increase per year). In decedents age 65 years or older, simpler forms of CHD like shunt lesions became more prominent contributors, accounting for 46% of deaths.

Conclusion: Rates of mortality due to ACHD have declined significantly for adults with CHD, however, continued mortality due to single ventricle physiology remains an area requiring improved strategies to increase survival.