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. 2025 Jun;4(6 Pt 1):101812.
doi: 10.1016/j.jacadv.2025.101812. Epub 2025 May 19.

Longitudinal Trends in Pediatric Survival by Congenital Heart Defect in Texas, 1999 to 2017

Sara B Stephens  1 , Shaine A Morris  2 , Renata H Benjamin  3 , Mark A Canfield  3 , Charles J Shumate  4 , Ruosha Li  5 , Cecilia Cazaban-Ganduglia  6 , A J Agopian  7
Affiliations

Longitudinal Trends in Pediatric Survival by Congenital Heart Defect in Texas, 1999 to 2017

Sara B Stephens et al. JACC Adv. 2025 Jun.

Abstract

Background: Despite previously improved survival among children with congenital heart defects (CHDs), U.S. population-level evaluations of survival within recent years are scarce.

Objectives: The purpose of this study was to describe the survival landscape among children with CHDs in a large population-based birth defects registry overall and by CHD lesion.

Methods: This population-based cohort study evaluated 1999 to 2017 live births with ≥1 major CHD in the statewide Texas Birth Defects Registry. Variables included CHD lesion, demographics, gestational age at birth (term/preterm), low birthweight (<2,500 g at birth), among others. Kaplan-Meier analyses were used to describe survival to 7 days, 28 days, 1 year, 5 years, and 10 years of life. Kaplan-Meier survival estimates were generated for 1-year survival for CHDs overall by lesion, using log-rank tests assessing differences by exposure.

Results: Of 61,656 children with CHDs, survival was 98.1% and 90.7% at 7 days and 10 years, respectively, and substantially varied by lesion (range, 50.0% to 97.3% 10-year survival). Survival longitudinally improved for complex lesions including hypoplastic left heart syndrome (48.7% 1-year survival for cases born 1999-2004 vs 64.8% in 2014-2017; P < 0.0001). One-year survival differed by maternal race/ethnicity (eg, 58.3% for cases with complex pulmonary atresia born to non-Hispanic Black mothers vs 80.5% for non-Hispanic White mothers, P = 0.01), sex, gestational age, birthweight, and extracardiac defect status.

Conclusions: One-year survival improved for most CHDs over recent decades, although survival varies widely by CHD and characteristics. Findings have implications for clinical counseling, population-level resource and research planning, and reinforce the need for mitigation of disparities among individuals with CHDs.

Keywords: congenital heart disease; double outlet right ventricle; hypoplastic left heart syndrome; mortality; time trends; tricuspid atresia.

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Conflict of interest statement

Funding support and author disclosures This project was supported by a grant from the Eunice Kennedy Shriver National Institute of Child Health & Human Development (R01 HD093660) and the Centers for Disease Control and Prevention (CDC). Support for data collection was provided in part by the Maternal and Child Health Section, Texas Department of State Health Services, using Title V Maternal and Child Health Block Grant funds. This study was funded in part by a CDC birth defects surveillance cooperative agreement with the Texas Department of State Health Services (HHS 00096260001) and Health Resources and Services Administration (HRSA) Block Grant funds. The contents are those of the author(s) and do not necessarily represent the official views of, nor an endorsement, by HRSA or CDC. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Central Illustration
Central Illustration
Longitudinal Trends and Survival Disparities Among >60,000 Infants With Congenital Heart Defects (A) Overall survival in children with CHD has markedly increased over the last 2 decades, with the largest improvements observed for infant survival. (B) While infant survival varied by CHD lesion type and was highest among those with critical CHDs, racial and ethnic disparities in infant survival persisted with the lowest survival often observed in non-Hispanic Black and Hispanic infants. CHD = congenital heart defect; other abbreviation as in Figure 3.
Figure 1
Figure 1
Percentage of Deaths Among Infants With Congenital Heart Defects, Texas, 1999 to 2017 Births AVSD = atrioventricular septal defect; DILV = double inlet left ventricle; DORV = double outlet left ventricle; GA = great arteries; IVS = intact ventricular septum; VSD = ventricular septal defect.
Figure 2
Figure 2
Kaplan-Meier Survival Estimates Among Infants With Congenital Heart Defect by Birth Year, Texas, 1999 to 2017 Births
Figure 3
Figure 3
Kaplan-Meier Survival Estimates Among Infants With Congenital Heart Defect by Maternal Race/Ethnicity, Texas, 1999 to 2017 Births NH = non-Hispanic.
Figure 4
Figure 4
Kaplan-Meier Survival Estimates Among Infants With Congenital Heart Defect by Infant Sex, Texas, 1999 to 2017 Births
See this image and copyright information in PMC

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