Background: Myocardial performance index (MPI) was reported as a parameter of ventricular systolic and diastolic function, as well as a useful tool to predict the outcome in patients with ventricular dysfunction. Hypothesis: To compare MPI with classical echocardiographic parameters as an independent marker of death in children with idiopathic dilated cardiomyopathy (IDCM). Methods: Fifty-five children (13 deaths) underwent 104 echocardiograms from January 1996 to May 2005. Right ventricle (RV) MPI and left ventricle (LV) MPI, and 9 classical echocardiographic parameters (left atrium [LA]/body surface area [BSA], distance between mitral E point and ventricular septum, LV mass/body surface area, RV shortening fraction, LV end-systolic and end-diastolic dimensions/body surface area, LV ejection fraction, fiber circumferential shortening velocity, and mitral deceleration time) were compared. Statistical analysis was performed by chi-square, Pearson's correlation and Student t-test, Kaplan-Meier method, Cox's method, and receiver operating curve (ROC). Statistical significance was considered with α<0.05 and p = 0.80. Results: Univariate analysis showed that all studied parameters were markers of death. There was a high correlation between RVMPI and LVMPI (r = 0.847-p = 0.0001); therefore, to avoid bias, RVMPI was discharged from multivariate analysis. In the deceased group, moderate/severe mitral regurgitation was frequent (76.9%; confidence interval [CI] 95% = 46.2%-94.9%) and it was considered in multivariate analysis. In Cox's multivariate analysis, LVMPI was the only independent marker of death (p = 0.0213). The ideal cut-off was 0.63 with 92.3% sensitivity, 66.7% specificity, and fitted ROC area = 0.918. Conclusions: In children with IDCM, LVMPI is an independent marker of death.

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 weighed 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. ResumoObjetivos: A desnutrição é marcadora independente de óbito na cardiomiopatia dilatada idiopática. Foi analisada a repercussão da introdução da L-carnitina nos parâmetros nutricionais e ecocardiográ-ficos em crianças com cardiomiopatia dilatada idiopática.Métodos: Estudo prospectivo aberto de 11 crianças, comparadas com 40 controles, pareados para sexo e idade. Foi administrada L-carnitina oral (100 mg/kg/dia), além do tratamento padrão. Foram realizadas 118 pesagens no grupo L-carnitina e 264 nos controles, além de 65 ecocardiogramas no grupo L-carnitina e 144 nos controles. Análise estatística: qui-quadrado, teste t de Student, ANOVA e correlação de Pearson. Foi utilizado alfa = 0,05.Resultados: Grupo L-carnitina: idade = 3,82 anos, 72,7% (p = 0,033) menores de 2 anos e do sexo feminino, e 90,9% (p = 0,001) em classe funcional III e IV. Não ocorreram óbitos no período. Não houve diferença no percentil de peso inicial (31,2±8,74 vs. 19,6±21,2) (p = 0,29) nem no índice z (-0,68±1,05 vs. -1,16±0,89) (p = 0,24). Ocorreu aumento do percentil (p = 0,026) e...

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 weighed 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 (

The analysis of critical end diastolic left ventricular volume (EDLVV) can add another parameter in the indication of univentricular correction in patients with post-valvotomy aortic stenosis, during the first year of life.

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