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High-risk single ventricle palliation in children with Down syndrome: single institution experience
Yinn K Ooi1, Pranava Sinha2, Marcin Gierdalski2
11Department 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.
Insights
Outcomes for children with Down syndrome undergoing single ventricle palliation are poor, especially those with high-risk factors. This study highlights the critical need for further research into managing these complex cardiac cases.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Genetics
Background:
- 40-50% of children with Down syndrome have cardiac defects, often treatable with biventricular repair.
- Single ventricle palliation outcomes are improving, but data on Down syndrome patients with high-risk factors are limited.
Purpose of the Study:
- To investigate the outcomes of children with Down syndrome and high-risk factors undergoing single ventricle palliation.
Main Methods:
- Retrospective study of Down syndrome patients on the single ventricle palliation pathway (2005-2011).
- Review of operative, clinical, echocardiographic, haemodynamic, and follow-up data.
Main Results:
- Eight Down syndrome patients underwent single ventricle palliation; five had high-risk factors.
- Mortality was 80% in the high-risk group versus 33% in the non-high-risk group.
- Overall survival was 37.5% after a median follow-up of 138 days.
Conclusions:
- The prognosis for Down syndrome patients with high-risk factors undergoing single ventricle palliation remains poor.
- Further multicenter, long-term studies are necessary to understand the cumulative impact of negative prognostic factors in this population.
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.
Conclusion:
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.
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