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