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

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

Objective: 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.