title: Survival in Children With Down Syndrome Undergoing Single-Ventricle Palliation.
abstract: BACKGROUND
  We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality.
  
  
  METHODS
  All patients with DS and single-ventricle anatomy documented in the electronic medical record at our institution from January 1, 1992, to May 1, 2014, were compared with patients with unbalanced atrioventricular septal defects and single-ventricle anatomy, without DS or heterotaxy, during the same period. Survival analysis was performed to evaluate factors associated with mortality, including the presence of DS.
  
  
  RESULTS
  There were 28 patients with DS and 30 patients without DS. One-year survival with DS was 75% (95% CI: 55% to 87%); 5-year survival was 61% (95% CI: 40% to 76%). All DS deaths except one occurred before 2 years of age. One-year non-DS survival was 93% (95% CI: 76% to 98%); 5-year survival was 85% (95% CI: 64% to 94%). Factors associated with death by univariable analysis included DS (p = 0.04), pulmonary vascular resistance (PVR) of at least 3 Wood units × meter(2) (WUm(2)) in the first year of life (p = 0.03), and moderate-to-severe atrioventricular valve regurgitation (p = 0.1). In combined analysis, when accounting for PVR of at least 3 WUm(2) (hazard ratio [HR] 9.8, 95% CI: 1.1 to 83.5, p = 0.04), DS was not associated with increased mortality (HR 1.5, 95% CI: 0.3 to 7.8, p = 0.66). No patient with DS with PVR less than 3 WUm(2) died.
  
  
  CONCLUSIONS
  Children with DS and single-ventricle anatomy have excellent survival when PVR is less than 3 WUm(2) in the first year of life, with minimal mortality beyond 2 years of age. When accounting for PVR, DS alone is not associated with increased mortality in patients with single-ventricle anatomy.
authors: Division of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
authors: John L
authors: JL
authors: Colquitt
authors: Division of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
authors: Shaine A
authors: SA
authors: Morris
authors: Division of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
authors: Susan W
authors: SW
authors: Denfield
authors: Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
authors: Charles D
authors: CD
authors: Fraser
authors: Division of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
authors: Yunfei
authors: Y
authors: Wang
authors: Division of Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas. Electronic address: wbkyle@bcm.edu.
authors: W Buck
authors: WB
authors: Kyle
mesh_terms: D000402:Airway Obstruction / Q000209:etiology / Q000601:surgery; D004314:Down Syndrome / Q000401:mortality*; D005260:Female; D006330:Heart Defects, Congenital / Q000235:genetics; D006343:Heart Septal Defects / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities / Q000601:surgery*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality; D016016:Proportional Hazards Models; D012372:ROC Curve; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate; D016896:Treatment Outcome; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents