Lesion-Specific Congenital Heart Disease Mortality Trends in Children: 1999 to 2017

Abstract

OBJECTIVE: Congenital heart disease (CHD) is a leading cause of premature death in infants and children. Currently limited data are available regarding lesion specific mortality over time. Our study aimed to describe pediatric mortality trends by CHD lesion in the United States. METHODS: We conducted a 19 year analysis (1999 to 2017) of publicly available, deidentified multiple cause of death data compiled and produced by the National Center for Health Statistics. Analysis was stratified by CHD diagnosis and age using 3 age categories (infants, 1 to 4 years, and 5 to 17 years). Temporal trends of CHD mortality and the effect of contributing risk factors were analyzed by using joinpoint regression. RESULTS: Mortality was highest for in infants for all CHD lesions, in particular for total anomalous pulmonary venous return. Significant declines in infant CHD mortality occurred for most other lesions. Contributing risk factors, including prematurity, extracardiac birth defects, and genetic conditions, occurred in 19% of infant CHD deaths and demonstrated worse mortality trends in the majority of lesions. Mortality rates remained highest for single ventricle lesions in all ages, with an infant mortality rate plateau in the later half of the study and progressive increasing mortality rates for children 5 to 17 years. CONCLUSIONS: CHD mortality is decreasing for most lesions. Because of the heterogenicity of CHD lesions, there is expected variability in mortality trends by lesion and age group. Single ventricle lesions continue to contribute most heavily to premature death because of CHD demonstrated by significant increases in mortality rate for children aged 5 to 17 years.

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. 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).…”

“…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.…”

Human milk feeding and direct breastfeeding improve outcomes for infants with single ventricle congenital heart disease: Propensity score matched analysis of the NPC-QIC registry

“…These infants undergo 3 staged palliative surgeries and/or catheterbased interventions and experience risk for morbidity (eg, necrotizing enterocolitis (NEC)), 1 developmental delay, 2 and family maladaptation 3,4 while incurring the highest hospital costs among United States (US) birth defects. 5 Mortality rates have been reduced by up to 38% over the past four decades; [6][7][8] yet, there remain opportunities to improve outcomes related to development and quality of life. 9 Feeding for infants with SV CHD is one such developmental area in need of improvement.…”