The role of L-carnitine in pediatric cardiomyopathy.
Metabolic and genetic factors underlie some forms of cardiomyopathy in childhood. A variety of inborn errors of metabolism can impair mitochondrial energy production, or beta-oxidation, in the heart and lead to myocardial dysfunction. L-Carnitine, an essential element of beta-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.
Winter
S
S
Valley Children's Hospital, Fresno, CA. 93703, USA.
Jue
K
K
Prochazka
J
J
Francis
P
P
Hamilton
W
W
Linn
L
L
Helton
E
E
D009202:Cardiomyopathies / Q000097:blood / Q000378:metabolism*; D002331:Carnitine / Q000097:blood / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D014804:Vitamin B Deficiency / Q000097:blood
D002331:Carnitine