Narrowing the Survival Gap: Trends in Survival of Individuals with Down syndrome with and without Congenital Heart Defects Born 1979 – 2018

Details

• Alternative Title:
  J Pediatr
• Personal Author:
  Wright, Lydia K. ; Stallings, Erin B. ; Cragan, Janet D. ; Pabst, Laura J. ; Alverson, CJ ; Oster, Matthew E.
• Description:
  Objective: ; To evaluate the hypothesis that childhood survival for individuals with Down syndrome (DS) and congenital heart defects (CHDs) has improved in recent years, approaching survival of those with DS without CHDs. ; Study design: ; Individuals with DS born 1979–2018 were identified through the Metropolitan Atlanta Congenital Defects Program, a population-based birth defects surveillance system administered by the Centers for Disease Control and Prevention. Survival analysis was performed to evaluate predictors of mortality for those with DS. ; Results: ; The cohort included 1,671 individuals with DS; 764 had associated CHDs. Five-year survival in those with DS with CHD improved steadily among individuals born in the 1980s through the 2010s (85% to 93%, p=0.01) but remained stable (96% to 95%, p=0.97) in those with DS without CHDs. The presence of a CHD was not associated with mortality through 5 years of age for those born 2010 or later (hazard ratio 2.63 95% confidence interval 0.95 – 8.37). In multivariable analyses, atrioventricular septal defects were associated with early (<1 year) and late (>5 year) mortality, while ventricular septal defects were associated with intermediate (1–5 years) mortality and atrial septal defects with late mortality, when adjusting for other risk factors. ; Conclusions: ; The gap in five-year survival between children with DS with and without CHDs has improved over the last four decades. Survival after 5 years remains lower for those with CHDs, although longer follow-up will be needed to determine if this difference lessens for those born in the more recent years.
• Subjects:
  • Child
  • Humans
  • Down Syndrome
  • Heart Defects, Congenital
  • Heart Septal Defects
  • Heart Septal Defects, Atrial
  • Heart Septal Defects, Ventricular
• Source:
  J Pediatr. 260:113523
• Pubmed ID:
  37244582
• Pubmed Central ID:
  PMC10538432
• Document Type:
  Journal Article
• Funding:
  CC999999/ImCDC/Intramural CDC HHSUnited States/
• Volume:
  260
• Download URL:
  https://stacks.cdc.gov/view/cdc/133530/cdc_133530_DS1.pdf
• File Type:
  [PDF - 699.33 KB]
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha-512:418d5720a15fced6da311a2928065b764aa9bb89581d6294097e7ea74197f75d9cdc0c0eb2a63b9649083a06884a1aa6d89bf763f3ca47b23c3fd2b8b05f8bd2