CARNITINE DEFICIENCY-INDUCED CARDIOMYOPATHY

The results of clinical and animal studies suggest that a short term p eriod of moderate secondary carnitine deficiency, in and of itself, do es not have a major effect on the cardiac contractile function, althou gh substrate oxidation may be altered. However, with longer durations of carnitine deficiency alterations occur within the heart that may re sult in impaired contractile performance, particularly at high workloa ds. At this point, the mechanisms responsible for the cardiac depressi on are uncertain. We hypothesize that the alterations in substrate met abolism produced by the carnitine deficient state results in inadequat e ATP production under high workload conditions which result in impair ed cardiac contractile performance. Carnitine deficiency may also indu ce a number of changes in gene expression of key enzymes required for normal cardiac contractile function and metabolism.