静脉注射左卡尼汀治疗肺动脉高压并右心功能障碍疗效观察

Clinical efficacy of intravenous L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension

目的观察静脉注射左卡尼汀治疗肺动脉高压导致右心功能障碍的疗效。方法入6例WHO心功能分级Ⅲ－级的特发性肺动脉高压14例、先天性心脏病相关性肺动脉高压36例及结缔组织病相关性肺动脉高压16例患者，分为左卡尼汀治疗组40例和对照组26例。左卡尼汀治疗组在常规治疗基础上给予左卡尼汀5g／d，静脉滴注，连用7d。分别于研究开始及7d后进行6min步行距离、WHO心功能分级、体格检查及血清标记物检查，评价两组疗效和记录不良反应。结果治疗前后比较，左卡尼汀组患者的6min步行距离增加75m，对照组增加45m（P=0．04）。左卡尼汀组16例心功能改善2级，13例改善1级，6例无变化，5例恶化一级以上；对照则分别为心功能改善2级3例，改善1级8例，无变化9例和恶化一级以上6例（P=0．04）。左卡尼汀组B型利钠肽水平下降58．16ng／L，而对照组下降33．29ng／L（P=0．01）。左卡尼汀组收缩压升高8．1mmHg（1mmHg=0．133kPa），对照组升高2．4mmHg（P=0．03）。无患者因严重不良反应退出研究。结论常规治疗的基础上联合应用左卡尼汀，可改善肺动脉高压所导致的右心功能障碍患者的运动耐量和心功能分级，并且安全性、耐受性良好。

Objective To investigate the efficacy of L-carnitine in patients with right-sided heart failure induced by pulmonary arterial hypertension （PAH）. Methods A total of 66 pulmonary arterial hypertension patients （ 14 idiopathic, 36 congenital heart disease associated and 16 connective-tissue disease associated PAH, WHO heart functional class m, n = 38 or n = 28 ） were enrolled in this study and divided into control group （26 cases） and L-carnitine group （40 cases）. All patients received conventional treatment according to guideline for treatment of right-sided heart failure. The patients in L-carnitine group received 5 g/d L-carnitine intravenously for seven days. Six-minute walking distance, WHO heart functional class, physical examination, and serum markers were evaluated at baseline and 7 days after enrollment. Results Compared to the baseline, six-minute walking distance was significantly increased （75 m vs. 45 m, P 〈 0.05 ） , WHO heart functional class significantly improved （ improved 2 classes in 16 patients, improved 1 class in 13, no improved in 6, worsen in 5 vs. 3, 8, 9, 6 respectively in the control, P =0. 04）, BNP level significant decreased （58. 16 ng/L vs. 33. 29 ng/L, P = 0. 01 ） and systolic blood pressure significantly increased [8. 1 mm Hg vs. 2.4 mm Hg（1 mm Hg =0. 133 kPa）, P=0.03] in L- carnitine group compared with those in control group. No patient was withdrawn from this study for safety reasons. Conclusions L-carnitine could improve short-term exercise capacity and WHO heart functional class in right-sided heart failure patients induced by PAH.