Maternal race/ethnicity and survival experience of children with congenital heart disease

J Pediatr. 2013 Nov;163(5):1437-42.e1-2. doi: 10.1016/j.jpeds.2013.06.084. Epub 2013 Aug 6.


Objective: To investigate the existence of racial/ethnic disparity in mortality risk among children with individual congenital heart defects and identify any other risk factors.

Study design: The study cohort, comprising children born between 1983 and 2006 with a selected congenital heart defect, was matched to death records to ascertain vital status. The birth and maternal risk factors were obtained from birth certificates.

Results: After adjusting for covariates using a multivariate regression model, the risk of mortality was significantly higher in children of non-Hispanic black mothers with transposition of the great arteries (hazard ratio (HR), 1.31; 95% CI, 1.07-1.60), tetralogy of Fallot (HR, 1.34; 95% CI, 1.06-1.69), and coarctation of the aorta (HR, 1.40; 95% CI, 1.10-1.79), compared with children of non-Hispanic white mothers. Time trends analysis examining the mortality risk by survival age and birth period found a significant decrease in 5-year mortality risk from 1983 to 2003 births, with a nearly 50% reduction for hypoplastic left heart syndrome and coarctation of the aorta across 3 maternal racial/ethnic groups examined.

Conclusion: Our findings may help identify at-risk populations and mortality risk factors and thereby contribute to improved survival and quality of life for these children across the lifespan.

Keywords: CA; CHD; CMR; Coarctation of the aorta; Congenital Malformations Registry; Congenital heart defect; HLHS; HR; Hazard ratio; Hypoplastic left heart syndrome; ICD-9-CM; International Classification of Diseases, Ninth Revision, Clinical Modification; NH; Non-Hispanic; TGA; TOF; Tetralogy of Fallot; Transposition of the great arteries; VSD; Ventricular septal defect.

MeSH terms

  • Adolescent
  • Adult
  • African Americans
  • Aortic Coarctation / ethnology
  • Child
  • Child, Preschool
  • Cohort Studies
  • Disease Susceptibility
  • European Continental Ancestry Group
  • Female
  • Heart Defects, Congenital / ethnology*
  • Heart Defects, Congenital / mortality
  • Hispanic Americans
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Mothers*
  • Multivariate Analysis
  • Proportional Hazards Models
  • Risk Factors
  • Transposition of Great Vessels / ethnology
  • Treatment Outcome
  • Young Adult