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Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy

Published online by Cambridge University Press:  20 August 2009

Narendra R. Dereddy ,
David Kronn  and
Usha Krishnan
Narendra R. Dereddy
Affiliation:
Department of Pediatrics, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
David Kronn
Affiliation:
Department of Metabolic and Genetic Disorders, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
Usha Krishnan*
Affiliation:
Department of Pediatric Cardiology, Maria Fareri Children’s Hospital, Westchester Medical Center, Valhalla, New York, United States of America
*
Correspondence to: Usha Krishnan, MD, Division of Pediatric Cardiology, 618, Munger Pavilion, New York Medical College, Valhalla, NY 10595, United States of America. Tel: 1-914-493-8372; Fax: 914-594-4513; E-mail: usha_krishnan@nymc.edu
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Abstract

Inborn errors of fatty acid metabolism are important causes of reversible cardiomyopathy in infancy. Disorders in long chain fatty acid oxidation can lead to cardiomyopathy, as fatty acid beta oxidation is the major source of myocardial energy after birth. We present 2 cases of such disorders with cardiac manifestations during infancy, which responded well to a diet low in long chain fatty acids.

Keywords

Heart failurelipid metabolismpericardial effusion
Type
Brief Report
Information
Cardiology in the Young , Volume 19 , Issue 5 , October 2009 , pp. 540 - 542
Copyright
Copyright © Cambridge University Press 2009

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