Objectives: Malnutrition is an independent predictor of death in idiopathic dilated cardiomyopathy. An analysis was performed of the impact of L-carnitine supplementation on the nutritional status and echocardiogram parameters of children with idiopathic dilated cardiomyopathy. Methods: This was an open label cohort of 11 patients who received L-carnitine (100 mg/kg/day) plus the conventional medical treatment, compared with 40 controls, matched for gender and age. The L-carnitine group was

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... hed 118 times and the controls 264 times. Additionally, the L-carnitine group underwent 65 two-dimensional echocardiograms and the controls 144. Chi-square, Students t test, Pearson correlation and ANOVA were calculated with alpha = 0.05. Results: For the L-carnitine group: age at presentation = 3.82 years old, 72.7% (p = 0.033) were females younger than 2 years and 90.9% (p = 0.0001) were in functional classes III and IV. There were no deaths during this period. At presentation, no differences were observed in weight percentile (31.2±8.74 vs. 19.6±21.2) (p = 0.29) or z score (-0.68±1.05 vs. 1.16±0.89) (p = 0.24). Increases were observed in both the percentile (p = 0.026) and z score (p = 0.033) after the introduction of L-carnitine. At presentation, there were no differences in ejection fraction (54.9%±3.8 vs. 49.3%±6.6) (p = 0.19), but LV mass/ BSA were greater in the L-carnitine group (169.12 g/m 2 ±26.24 vs. 110.67 g/m 2 ±15.62) (p = 0.0005). After the introduction of L-carnitine an increase in ejection fraction (48.3±7 to 67.2±7) (p = 0.044) was observed. LV mass/BSA decreased (164.29 g/m 2 ±28.14 to 110.88 g/m 2 ±28.88), but without significance (p = 0.089) Conclusion: In children with idiopathic dilated cardiomyopathy, supplementation of L-carnitine may be helpful for nutritional and echocardiographic improvement. J Pediatr (Rio J). 2005;81(5):368-72: Acetylcarnitine, congestive cardiomyopathy, child, malnutrition, congestive heart failure, echocardiography. Artigo submetido em 28.01.05, aceito em 25.05.05. Como citar este artigo: Azevedo VM, Albanesi Filho FM, Santos MA, Castier MB, Cunha MO. O papel da L-carnitina no estado nutricional e na evolução ecocardiográfica da cardiomiopatia dilatada idiopática da infância. J Pediatr (Rio J). 2005;81:368-72.