Survival in Children With Down Syndrome Undergoing Single-Ventricle Palliation.

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.