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. 2022 Aug 1;114(13):725-745.
doi: 10.1002/bdr2.2044. Epub 2022 May 20.

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

Melodie M Lynn  1   2 , Jason L Salemi  3   4 , Callie J Meath  2 , Stephen J Dolgner  1   2   5 , Shaine A Morris  1   2 , S Kristen Sexson Tejtel  1   2 , Keila N Lopez  1   2
Affiliations

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

Melodie M Lynn et al. Birth Defects Res. .

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.

Keywords: Fontan procedure; congenital heart defects; epidemiology; health care outcome assessment.

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Figures

FIGURE 1
FIGURE 1
Joinpoint Adult Mortality for All Congenital Heart Disease Diagnoses from 1999 to 2017. Includes all adults, defined as 18 year or greater at time of death, with congenital heart disease listed as the underlying cause of death. * indicates that the Annual Percent Change (APC) is significantly different from zero at the alpha = 0.05 level
FIGURE 2
FIGURE 2
Joinpoint Ventricular Septal Defect Mortality from 1999 to 2017. Includes all adults, defined as 18 year or greater at time of death, with ventricular septal defect listed as the underlying cause of death. * indicates that the Annual Percent Change (APC) is significantly different from zero at the alpha = 0.05 level
FIGURE 3
FIGURE 3
Joinpoint Atrial Septal Defect Mortality from 1999 to 2017. Includes all adults, defined as 18 year or greater at time of death, with atrial septal defect listed as the underlying cause of death. * indicates that the Annual Percent Change (APC) is significantly different from zero at the alpha = 0.05 level
FIGURE 4
FIGURE 4
Joinpoint Other Specified Congenital Heart Disease Mortality from 1999 to 2017. Includes all adults, defined as 18 year or greater at time of death, with one of the ICD-10 codes from the composite group, other specified congenital heart disease, listed as the underlying cause of death. * indicates that the Annual Percent Change (APC) is significantly different from zero at the alpha = 0.05 level
FIGURE 5
FIGURE 5
Joinpoint Tetralogy of Fallot Mortality from 1999 to 2017. Includes all adults, defined as 18 year or greater at time of death, with tetralogy of Fallot listed as the underlying cause of death. * indicates that the Annual Percent Change (APC) is significantly different from zero at the alpha = 0.05 level
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