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High-risk single ventricle palliation in children with Down syndrome: single institution experience

Published online by Cambridge University Press:  25 March 2014

Yinn K. Ooi
Department 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
Pranava Sinha
Department of Cardiovascular Surgery, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
Marcin Gierdalski
Department of Cardiovascular Surgery, Children’s National Health System, The George Washington University School of Medicine, Washington, District of Columbia, United States of America
Ashraf Harahsheh*
Department 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
Correspondence to: Dr A. Harahsheh, MD, FACC, FAAP, Assistant Professor, Department of Pediatric Cardiology, George Washington University Center for Heart, Lung and Kidney Disease, Children’s National Health System 111 Michigan Ave, N.W. Washington, DC 20010, United States of America. Tel: 202 476 2020; Fax: 202 476 5700; E-mail:


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.

Original Articles
© Cambridge University Press 2014 

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Yinn K. Ooi and Ashraf Harahsheh were involved in the analysis of data and drafting of the manuscript. Marcin Gierdalski was involved in data gathering and analysis of data. Pranava Sinha provided critical revision of the manuscript for important intellectual content.


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