title: Congenital heart surgery outcomes in Down syndrome: analysis of a national clinical database.
abstract: OBJECTIVE
  We describe patient characteristics and postoperative morbidity and mortality rates for patients with Down syndrome undergoing congenital heart disease surgery.
  
  
  METHODS
  This retrospective cohort study used the Society of Thoracic Surgeons Congenital Heart Surgery Database to compare patient characteristics and postoperative outcomes for patients (0-18 years) with or without Down syndrome who underwent surgery in 2000-2008.
  
  
  RESULTS
  A total of 45,579 patients (4350 patients with Down syndrome and 41,229 without Down syndrome) were included (median age: 7 months [interquartile range [IQR]: 47 days to 4 years]; 56% male). Patients with Down syndrome were younger at surgery, with the exception of those undergoing tetralogy of Fallot repair or atrioventricular septal defect repair. Mortality rates for patients with or without Down syndrome did not differ significantly. Lengths of stay were prolonged for patients with Down syndrome undergoing atrial septal defect closure (median: 4 days [IQR: 3-5 days] vs 3 days [IQR: 2-4 days]; P < .0001), ventricular septal defect closure (median: 5 days [IQR: 4-8 days] vs 4 days [IQR: 3-6 days]; P < .0001), or tetralogy of Fallot repair (7 days [IQR: 5-10 days] vs 6 days [IQR: 5-9 days]; P < .001) and were associated with postoperative respiratory and infectious complications. Patients with Down syndrome undergoing ventricular septal defect closure had a higher rate of heart block requiring pacemaker placement (2.9% vs 0.8%; P < .0001).
  
  
  CONCLUSIONS
  In this large, contemporary cohort, Down syndrome did not confer a significant mortality risk for the most common operations; however, postoperative morbidity remained common.
authors.affiliation: Division of Cardiology, Department of Pediatrics, Duke University Medical Center, Durham, NC 27715, USA.
authors.forename: James C
authors.initials: JC
authors.lastname: Fudge
authors.forename: Shuang
authors.initials: S
authors.lastname: Li
authors.forename: James
authors.initials: J
authors.lastname: Jaggers
authors.forename: Sean M
authors.initials: SM
authors.lastname: O'Brien
authors.forename: Eric D
authors.initials: ED
authors.lastname: Peterson
authors.forename: Jeffrey P
authors.initials: JP
authors.lastname: Jacobs
authors.forename: Karl F
authors.initials: KF
authors.lastname: Welke
authors.forename: Marshall L
authors.initials: ML
authors.lastname: Jacobs
authors.forename: Jennifer S
authors.initials: JS
authors.lastname: Li
authors.forename: Sara K
authors.initials: SK
authors.lastname: Pasquali
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D004314:Down Syndrome / Q000453:epidemiology*; D005260:Female; D006330:Heart Defects, Congenital / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery*; D006343:Heart Septal Defects / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000401:mortality*; D015995:Prevalence; D012189:Retrospective Studies; D015996:Survival Rate; D014481:United States / Q000453:epidemiology