Pediatric cardiomyopathies related to fatty acid metabolism

Abstract Inborn errors of metabolism affecting the fatty acid ß-oxidation pathway lead to cardiomyopathy, arrhythmia, and sudden death. In this article, an overview of the clinical presentations, diagnosis, treatment, and long-term outcome is provided with an emphasis on cardiovascular manifestation...

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Bibliographic Details
Published inProgress in pediatric cardiology Vol. 25; no. 1; pp. 69 - 78
Main Authors Hill, Kevin D, Hamid, Rizwan, Exil, Vernat J
Format Journal Article
LanguageEnglish
Published Elsevier Ireland Ltd 01.04.2008
Subjects
Arrythmia
Cardimyopathy
Cardiovascular
Fatty acid oxidation
Metabolism
Pediatrics
Fatty acid oxidation
Arrythmia
Metabolism
Cardimyopathy
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Summary:Abstract Inborn errors of metabolism affecting the fatty acid ß-oxidation pathway lead to cardiomyopathy, arrhythmia, and sudden death. In this article, an overview of the clinical presentations, diagnosis, treatment, and long-term outcome is provided with an emphasis on cardiovascular manifestations. With the expanded newborn screening program, these patients are coming to clinical attention earlier and the opportunity for pre-symptomatic intervention exists. It is important that clinicians are familiar with the clinical presentation, diagnostic evaluation, and management of this clinically heterogeneous group of disorders.
ISSN:1058-9813
1558-1519
DOI:10.1016/j.ppedcard.2007.11.007