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

Abstract

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.

Survival From Birth Until Young Adulthood Among Individuals With Congenital Heart Defects: CH STRONG

“…Another recent publication also reported that all-cause death rates among Danish individuals with simple CHDs who survived to at least 5 years of age were similar to matched controls without CHDs until ≈70 years of age, at which point higher death is driven by excess risk of comorbidity. 33 In a previously published meta-analysis combining US and non-US mortality estimates, 34 Black race was found to Aside from CHD severity and maternal race and ethnicity, noncardiac anomalies 31,32 and low birth weight 12 have also been associated with reduced survival among individuals with CHDs in previous literature and in this analysis. Although earlier birth year has been identified as a risk factor for infant death among those with severe CHDs born between 1979 and 2005 in previous literature, 12 earlier birth years were only associated with increased infant mortality among those with nonsevere CHDs in the present analysis.…”

“…Aside from CHD severity and maternal race and ethnicity, noncardiac anomalies 31,32 and low birth weight 12 have also been associated with reduced survival among individuals with CHDs in previous literature and in this analysis. Although earlier birth year has been identified as a risk factor for infant death among those with severe CHDs born between 1979 and 2005 in previous literature, 12 earlier birth years were only associated with increased infant mortality among those with nonsevere CHDs in the present analysis.…”

“…11 Overall, severe CHDs are commonly associated with reduced survival in the first year of life 12,29,30 and into adulthood. 11,12 Furthermore, our analysis contributes to a body of literature concluding that overall survival is lowest specifically for those with hypoplastic left heart syndrome, 27,28,31,32 and survival after 1 year of age is lowest for all single-ventricle defects.…”

“…11 Overall, severe CHDs are commonly associated with reduced survival in the first year of life 12,29,30 and into adulthood. 11,12 Furthermore, our analysis contributes to a body of literature concluding that overall survival is lowest specifically for those with hypoplastic left heart syndrome, 27,28,31,32 and survival after 1 year of age is lowest for all single-ventricle defects. In a previous publication comparing patients up to 32 years after CHD surgery with the general population, 11 the authors found the overall SMR to be 8.3, and it remained elevated among those with nonsevere CHDs (who still required CHD surgery).…”