Plasma carnitine status - a prognostic factor in children with dilated cardiomyopathy

Summary Objective - Dilated cardiomyopathy is a rare disorder in childhood that results in a high mortality. The aim of our study was to evaluate the prognostic relevance of the individual plasma carnitine status in children with dilated cardiomyopathy.Methods - In 26 patients plasma carnitine concentrations were determined before and after 6 and 12 months of L-carnitine treatment. According to the plasma short chain acyl-carnitine/free carnitine ratio (AC/FC) at the first presentation children were divided into two groups.Results - In group 1 (AC/FC < 0.4) the median time from diagnosis until death was 35.8 months, the cumulative survival rate was 84% after 2 years. In group 2 (AC/FC > 0.4) median time from diagnosis until death was 8 months, the cumulative survival rate was 50% at 2 years (p < 0.05).Dividing both groups into survivors and nonsurvivors in group 2 a significantly higher AC/FC ratio in the nonsurvivors could be found (survivors 0.78 v 1.3 in nonsurvivors). A significant improvement of left ventricular function 6 and 12 months after presentation and after starting L-carnitine treatment could only be documented in the surviving patients of group 2.Conclusion - The individual plasma carnitine status in children with dilated cardiomyopathy may serve as a risk factor for survival.