L-carnitine is an amino acid involved in the mechanism by which fatty acids are transported through the mitochondrial wall for oxidation, To determine whether the in vivo metabolism of lipoprotein(a) (Lp[a]) is linked to intramitochondrial oxidative metabolism, we investigated the effects of exogenously administered L-carnitine in patients with high serum concentrations of Lp(a), Eighteen outpatients received open-label treatment with L-carnitine 3 g/d for 8 weeks; 10 patients continued treatment for up to 16 weeks, Serum Lp(a) levels were decreased from a mean baseline value of 72.71 mg/dL by a mean of 14% after 4 weeks and 15% after 8 weeks in 18 patients and 13% after 12 weeks and 12% after 16 weeks of treatment in 10 patients (P < 0.05 for all results), In a second phase of the study, 12 of the 18 patients completed an additional 20 weeks of treatment, Serum Lp(a) was reduced from a mean baseline value of 92.56 mg/dL by a mean of 5% at 4 weeks, 6% at 8 weeks, 8% at 12 weeks, 12% at 16 weeks, and 13% at 20 weeks (P < 0.05 for all results), Further investigation of L-carnitine appears warranted in patients with elevated Lp(a) levels.