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The Role of L-Carnitine in Pediatric Cardiomyopathy

Genetics/Metabolism, Valley Children's Hospital, 3151 North Millbrook, Fresno, CA

Kenneth Jue, MD

Valley Children's Hospital Fresno, CA

James Prochazka, MD

Valley Children's Hospital Fresno, CA

Paul Francis, MD

Geisinger Medical Center Danville, PA

Wade Hamilton, MD

University of Vermont Medical School, Burlington, VT

Lawrence Linn, MA

Metabolic Research and Analysis, Fresno, CA

Edward Helton, PhD

Sigma-Tau Pharmaceuticals, Gaithersburg, MD

Metabolic and genetic factors underlie some forms of cardiomyopathy in childhood. A variety of inborn errors of metabolism can impair mitochondrial energy production, or β-oxidation, in the heart and lead to myocardial dysfunction. L-Carnitine, an essential element of β-oxidation, transports fatty acids across the mitochondrial membrane for energy production. L-Carnitine deficiency syndromes are now well described as secondary to a variety of inborn errors of metabolism and often include cardiomyopathy in the clinical picture. Despite traditional therapies for cardiomyopathy, mortality for this disorder remains at well over 50%. Review of reports of L-carnitine supplementation studies and results from our own trial underscore the importance of its role in cardiac function and demonstrates that there is likely a subpopulation of patients with cardiomyopathy responsive to L-carnitine treatment. (J Child Neurol 1995; 10(Suppl):2S45-2S5 1).

Journal of Child Neurology, Vol. 10, No. 2 suppl, 2S45-2S51 (1995)
DOI: 10.1177/088307389501000207


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