Abstract:

Abstract： Objective To investigate the effect of exogenous L-carnitine on plasma free carnitine levels and cardiac function in children with cardiomyopathy. Methods A total of 88 patients with myocardiopathy were divided into treatment group and control group randomly. The control group received conventional therapy. The treatment group received additional intravenous L-carnitine therapy of 100mg/kg.d for 14 days on the basis of conventional therapy, then changed to oral L-carnitine consistently. The oral dosage was 10ml once a day in patients under 6 years of age，10ml twice a day in patients over 7 years.The plasma free carnitine levels and the improvement of cardiac function including left ventricular ejection fraction（LVEF），cardiac output（CO）， stroke volume （SV）， cardiac index （CI）， EF slope （MVEF） and left ventricular wall motion fast filling phase fraction （LFFPW），combined with electrocardiography， the plasma free carnitine levels， C-reactive protein （CRP）， myocardial enzymes were analyzed before and after therapy. Results Compared with control group， or those before treatment，the LVEF，CO，SV，CIMVEF and LFFPW of the treatment group improved significantly.The plasma free carnitine levels increased markly and had significant difference compared with those before treatment or control group. Conclusion L-carnitine supplement significantly increases the plasma free carnitine levels and improves cardiac function in children with cardiomyopathy.L-carnitine had a good therapeutic effect in children with cardiomyopathy.

Key words: L-carnitine, free carnitine levels, cardiac function;ejection fraction

摘要：

目的 探讨外源性左卡尼汀对心肌病（CM）患儿血浆游离肉碱水平及心功能的影响。方法 将2010年1月至2013年6月就诊于深圳市儿童医院的88例CM患儿，分为治疗组和对照组。对照组给予常规治疗。治疗组在常规治疗基础上缓慢静脉注射左旋卡尼汀100 mg/（kg·d）， 疗程14 d； 后左卡尼汀改为口服， 6岁以下10 mL/d（1.0 g/10mL），7岁以上每次10 mL，每日2次。观察两组患儿治疗前后血浆游离肉碱浓度和心功能改善情况，检测左室射血分数（LVEF）、 心排出量（CO）、 每搏输出量（SV）、 心脏指数（CI）； 左心舒张功能指标： EF 斜率（MVEF）和左室后壁运动快充盈期分数（LFFPW）；心电图、血浆游离肉碱浓度、血浆C反应蛋白（CRP）、心肌酶并进行比较。 结果　治疗组LVEF、C0、SV 、CIMVEF、LFFPW较治疗前和对照组治疗后明显提高。血浆游离肉碱水平较治疗前和对照组治疗后明显提高。结论 补充左卡尼汀可提高CM患儿游离肉碱浓度，改善心功能。

关键词: 左旋肉碱, 游离肉碱浓度, 心功能, 射血分数