Faculty, Staff and Student Publications

Language

English

Publication Date

6-1-2025

Journal

JACC: Advances

DOI

10.1016/j.jacadv.2025.101812

PMID

40393281

PMCID

PMC12149392

PubMedCentral® Posted Date

5-19-2025

PubMedCentral® Full Text Version

Post-print

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

Published Open-Access

yes

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