title: High-risk single ventricle palliation in children with Down syndrome: single institution experience.
abstract: BACKGROUND
  Of the children with Down syndrome 40-50% have cardiac defects and the majority of these cardiac defects are amenable to biventricular repair. The outcome of single ventricle palliation is improving; nonetheless, there are limited data on Down syndrome patients with associated high-risk factors undergoing single ventricle palliation. Our aim was to study the outcomes of children with Down syndrome and high-risk factors on the single ventricle palliation pathway.
  
  
  METHODS
  A retrospective study on all patients with Down syndrome on the single ventricle palliation pathway from 2005 until 2011 was conducted. Operative, clinical, echocardiographic, haemodynamic data, and follow-up data were reviewed.
  
  
  RESULTS
  A total of 310 patients underwent at least one single ventricle surgical intervention. Of those, eight patients had Down syndrome, five of which had associated risk factors - low birth weight, high pulmonary vascular resistance, pulmonary vein stenosis, significant atrioventricular valve regurgitation, and extracardiac anomalies. Mortality in the high-risk group was 80% (4/5), compared with 33% (1/3) in the non-high-risk patients. Overall, after a median follow-up period of 138 days (8-576 days), only 37.5% (3/8) of patients were alive.
  
  
  CONCLUSIONS
  Despite many improvements in the care of single ventricle patients, the fate of those with Down syndrome and associated high-risk factors remains poor. Further multicentre longer-term studies are needed to validate and quantify the cumulative effects of negative prognostic factors in this complex group of patients.
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Children's National Health System,The George Washington University School of Medicine,Washington,District of Columbia,United States of America.
authors.forename: Yinn K
authors.initials: YK
authors.lastname: Ooi
authors.affiliation: 2Department of Cardiovascular Surgery,Children's National Health System,The George Washington University School of Medicine,Washington,District of Columbia,United States of America.
authors.forename: Pranava
authors.initials: P
authors.lastname: Sinha
authors.affiliation: 2Department of Cardiovascular Surgery,Children's National Health System,The George Washington University School of Medicine,Washington,District of Columbia,United States of America.
authors.forename: Marcin
authors.initials: M
authors.lastname: Gierdalski
authors.affiliation: 1Department of Pediatrics,Division of Cardiology,Children's National Health System,The George Washington University School of Medicine,Washington,District of Columbia,United States of America.
authors.forename: Ashraf
authors.initials: A
authors.lastname: Harahsheh
mesh_terms: D018376:Cardiovascular Abnormalities / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality* / Q000601:surgery*; D004314:Down Syndrome / Q000150:complications* / Q000401:mortality / Q000473:pathology / Q000503:physiopathology; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D007230:Infant, Low Birth Weight; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care / Q000379:methods*; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014655:Vascular Resistance
keywords: pediatrics and congenital cardiac defect