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 Janu...

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Published inThe Annals of thoracic surgery Vol. 101; no. 5; pp. 1834 - 1841
Main Authors Colquitt, John L., MD, Morris, Shaine A., MD, MPH, Denfield, Susan W., MD, Fraser, Charles D., MD, Wang, Yunfei, PhD, Kyle, W. Buck, MD
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LanguageEnglish
Published Netherlands Elsevier Inc 01.05.2016
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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 × meter2 (WUm2 ) 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 WUm2 (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 WUm2 died. Conclusions Children with DS and single-ventricle anatomy have excellent survival when PVR is less than 3 WUm2 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.
AbstractList BACKGROUNDWe describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality.METHODSAll 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.RESULTSThere 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.CONCLUSIONSChildren 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.
We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality. 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. 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 × meter2 (WUm2) 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 WUm2 (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 WUm2 died. Children with DS and single-ventricle anatomy have excellent survival when PVR is less than 3 WUm2 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.
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 × meter2 (WUm2 ) 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 WUm2 (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 WUm2 died. Conclusions Children with DS and single-ventricle anatomy have excellent survival when PVR is less than 3 WUm2 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.
We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality. 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. 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. 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.
Author Fraser, Charles D., MD
Wang, Yunfei, PhD
Denfield, Susan W., MD
Kyle, W. Buck, MD
Colquitt, John L., MD
Morris, Shaine A., MD, MPH
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Snippet Background We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with...
We describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with mortality....
BACKGROUNDWe describe survival in patients with Down syndrome (DS) with single-ventricle anatomy and palliation and characterize risk factors associated with...
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SubjectTerms Airway Obstruction - etiology
Airway Obstruction - surgery
Cardiothoracic Surgery
Down Syndrome - mortality
Female
Heart Defects, Congenital - genetics
Heart Septal Defects - surgery
Heart Ventricles - abnormalities
Heart Ventricles - surgery
Humans
Hypertension, Pulmonary - drug therapy
Hypertension, Pulmonary - etiology
Infant
Kaplan-Meier Estimate
Male
Palliative Care
Postoperative Complications - mortality
Proportional Hazards Models
Retrospective Studies
Risk Factors
ROC Curve
Surgery
Survival Rate
Treatment Outcome
Vascular Resistance
Vasodilator Agents - therapeutic use
Title Survival in Children With Down Syndrome Undergoing Single-Ventricle Palliation
URI https://www.clinicalkey.es/playcontent/1-s2.0-S0003497515019554
https://dx.doi.org/10.1016/j.athoracsur.2015.11.047
https://www.ncbi.nlm.nih.gov/pubmed/26867706
https://search.proquest.com/docview/1783922017
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