Carnitine and its derivative propionyl-L-carnitine are endogenous cofactors which enhance carbohydrate metabolism and reduce the intracellular buildup of toxic metabolites in ischemic conditions. The carnitines have been, and are being used in a spectrum of diseases including multiple cardiovascular conditions. These include angina, acute myocardial infarction, postmyocardial infarction, congestive heart failure, peripheral vascular disease, dyslipidemia, and diabetes. Most published data on carnitine, propionyl-L-carnitine, and other carnitine congeners are favorable but the clinical trials have been relatively small. In currently used doses, these substances are virtually devoid of significant side effects.