Cardiomyopathy in childhood, mitochondrial dysfunction, and the role of L-carnitine

Cardiomyopathy in childhood is associated with high morbidity and mortality rates. Many metabolic causes have been identified, including genetic or ac quired defects in mitochondrial energy production affecting P-oxidation, ca rnitine transport, and the electron transport chain. Combining conventional inotropic and antiarrhythmic therapy with metabolic interventions has impr oved overall outcome. L-carnitine, a natural substance involved in mitochon drial transport of fatty acids, is one such therapy and plays a central rol e in the regulation of the inner mitochondrial supply of free coenzyme A. C arnitine deficiency can be caused by both genetic and environmental causes with resultant signs and symptoms of metabolic disease, including cardiomyo pathy. Administration of L-carnitine can result in improvement or resolutio n of the cardiomyopathy.