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

Abstract

Background: Limited population-based information is available on long-term survival of US individuals with congenital heart defects (CHDs). Therefore, we assessed patterns in survival from birth until young adulthood (ie, 35 years of age) and associated factors among a population-based sample of US individuals with CHDs.

Methods: Individuals born between 1980 and 1997 with CHDs identified in 3 US birth defect surveillance systems were linked to death records through 2015 to identify those deceased and the year of their death. Kaplan-Meier survival curves, adjusted risk ratios (aRRs) for infant mortality (ie, death during the first year of life), and Cox proportional hazard ratios for survival after the first year of life (aHRs) were used to estimate the probability of survival and associated factors. Standardized mortality ratios compared infant mortality, >1-year mortality, >10-year mortality, and >20-year mortality among individuals with CHDs with general population estimates.

Results: Among 11 695 individuals with CHDs, the probability of survival to 35 years of age was 81.4% overall, 86.5% among those without co-occurring noncardiac anomalies, and 92.8% among those who survived the first year of life. Characteristics associated with both infant mortality and reduced survival after the first year of life, respectively, included severe CHDs (aRR=4.08; aHR=3.18), genetic syndromes (aRR=1.83; aHR=3.06) or other noncardiac anomalies (aRR=1.54; aHR=2.53), low birth weight (aRR=1.70; aHR=1.29), and Hispanic (aRR=1.27; aHR=1.42) or non-Hispanic Black (aRR=1.43; aHR=1.80) maternal race and ethnicity. Individuals with CHDs had higher infant mortality (standardized mortality ratio=10.17), >1-year mortality (standardized mortality ratio=3.29), and >10-year and >20-year mortality (both standardized mortality ratios ≈1.5) than the general population; however, after excluding those with noncardiac anomalies, >1-year mortality for those with nonsevere CHDs and >10-year and >20-year mortality for those with any CHD were similar to the general population.

Conclusions: Eight in 10 individuals with CHDs born between1980 and 1997 survived to 35 years of age, with disparities by CHD severity, noncardiac anomalies, birth weight, and maternal race and ethnicity. Among individuals without noncardiac anomalies, those with nonsevere CHDs experienced similar mortality between 1 and 35 years of age as in the general population, and those with any CHD experienced similar mortality between 10 and 35 years of age as in the general population.

Keywords: adult; epidemiology; heart defects, congenital; mortality; survival.

Figure 1.. Survival for individuals with CHD born between 1980 and 1997 overall (A), by CHD severity (B), and by primary CHD diagnosis (C). CH STRONG.

AV indicates atrioventricular; CH STRONG, Congenital Heart Survey To Recognize Outcomes, Needs, and Well-Being; CHD, congenital heart defect; HLHS, hypoplastic left heart syndrome; Oth/Mult, other/multiple; Sh+V, shunt+valve; SV, single ventricle; TA – PS, tricuspid atresia without pulmonary stenosis; TA + PS, tricuspid atresia with pulmonary stenosis; TGA, transposition of the great arteries; and TOF, tetralogy of Fallot.

Figure 2.. Survival beyond ages 1, 10, and 20 years for individuals with CHDs born between 1980 and 1997. CH STRONG.

CH STRONG indicates Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being; and CHD, congenital heart defect.

Figure 3.. Survival beyond the first year of life for individuals with CHDs born between 1980 and 1997 by health and sociodemographic characteristics. CH STRONG.

AV indicates atrioventricular; CDT, county deprivation tertile; CH STRONG, Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being; CHD, congenital heart defect; dx, diagnosis; eth, ethnicity; HLHS, hypoplastic left heart syndrome; Mat, maternal; NH, non-Hispanic; NCA, noncardiac congenital anomalies; Oth/Mult, other/multiple; SV, single ventricle; Syn, syndromes; T, tertile; TA − PS, tricuspid atresia without pulmonary stenosis; TA + PS, tricuspid atresia with pulmonary stenosis; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and wgt, weight.

Figure 4.. Adjusted hazards ratios up to 35 years of age for individuals with CHDs born between 1980 and 1997, conditional on survival beyond the first year of life. CH STRONG multiple imputed data.

CH STRONG indicates Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being; CHD, congenital heart defect; NCA, noncardiac congenital anomalies; NH, non-Hispanic; and T, tertile. O denotes reference group. *Model includes CHD severity, NCA, birth year, site, sex, maternal race and ethnicity, and county deprivation tertile at birth. ^†Model includes NCA, birth year, site, sex, plurality, maternal race and ethnicity, and county deprivation tertile at birth. ^‡Model includes birth year and site. ^§Model includes sex and birth year. ^‖Model includes birth weight, NCA, birth year, site, sex, plurality, maternal race and ethnicity, and county deprivation tertile of birth county. ^#Model includes plurality, birth year, site, sex, and maternal race and ethnicity. **Model includes maternal race and ethnicity, birth year, and site. ^††Model includes county deprivation tertile of birth county, birth year, site, and maternal race and ethnicity. ^‡‡Model includes rurality of birth county, birth year, site, maternal race and ethnicity, and county deprivation tertile at birth.

Figure 5.. Standardized mortality ratios in the first year of life (A) and beyond the first year of life (B) for individuals with CHDs compared with age group-, sex-, year-, race and ethnicity–, and site-matched data from the general US population, CH STRONG, and National Vital Statistics System.

CH STRONG indicates Congenital Heart Survey to Recognize Outcomes, Needs, and Well-Being; and CHD, congenital heart defects. *All corresponding P values <0.001.