Potential usefulness of coenzyme Q10 in the treatment of idiopathic dilated cardiomyopathy in children

Abstract

It is well known that cardiac metabolism is under To our knowledge, there are no published data on direct control of the mitochondrial respiratory chain. the therapeutic efficacy of ubiquinone in cases of Coenzyme Q10 (ubiquinone) is a micronutrient uti- childhood dilated cardiomyopathy. Since this vita- lized by mitochondria in electron transport within the min-type substance produces no obvious side-effects, respiratory chain and thereby has a significant effect by means of non-invasive methods, the potential upon oxidative phosphorylation as well as intramito- therapeutic effect of ubiquinone upon clinical con- chondrial energy provision. Coenzyme Q10 has been dition and cardiac function was closely examined in shown to be deficient in myocardial tissue biopsies six children (age ranging from 2 months to 11 years) taken from dilated cardiomyopathy hearts, compared with idiopathic dilated cardiomyopathy. Each child to normal heart (1,2). had a clinical picture of congestive heart failure, Dilated cardiomyopathy is reported to be the most whereby the diagnosis of idiopathic dilated car- common form of cardiomyopathy, the diomyopathy had been established by excluding other aetiopathogenesis of which is still unknown, though aetiology. After medical therapy for heart failure, the most authors agree that in the vast majority of children were stabilized. patients it is probably the result of previous viral Ubiquinone was administered at a dose of 10 myocarditis (3). Therefore, one may suppose that mg / kg per day in divided two or three doses with dilated cardiomyopathy is caused by cellular bioener- soy-bean oil to ensure adequate absorption. Con- getic activity damage secondary to myocarditis and servative therapy with digitalis, diuretics, and ACE by compromise of oxidative phosphorylation. It is inhibitors were continued unchanged during the reported that in case of idiopathic dilated car- study. The clinical data and echocardiographic find- diomyopathy the myocardial concentration of ubi- ings of our patients are shown in Table 1. There was quinone measured in endomyocardial biopsies de- a significant increase in fractional shortening and clined in relation to the severity of the disease (4). ejection fraction in five cases after a mean period of Therefore, administration of ubiquinone might induce 866.32 months of coenzyme Q10 therapy (Table 1). an increase in the myocardial concentration of high- The fractional shortening was significantly improved energy phosphates.