卡维地洛治疗小儿原发性心内膜弹力纤维增生症疗效分析

Therapeutic effects of carvedilol on children with primary endocardial fibroelastosis

目的回顾性分析卡维地洛治疗小儿原发性心内膜弹力纤维增生症(EFE)疗效及相关影响因素。方法本研究收集自1990年1月至2012年12月在首都儿科研究所附属儿童医院心内科住院治疗,临床诊断为EFE的患儿46例,其中男22例,女24例,年龄2个月至4岁8个月,平均(6.2±3.5)个月。其中对照组(n=25)予肾上腺皮质激素、洋地黄及利尿剂治疗,治疗组(n=21)在对照组药物基础上加用卡维地洛。分别于治疗前、治疗后6个月、12个月时评价心脏大小、心脏功能、死亡率、不良反应等。结果 46例患儿中死亡5例,对照组4例,治疗组1例。治疗12个月后,治疗组左心室舒张末内径较治疗前明显降低[(41.7±6.5)mm vs.(47.0±6.7)mm;P<0.05];治疗6个月、12个月后左心室射血分数(EF)及短轴缩短率(FS)均较治疗前明显升高[EF:(55.6±13.2)%,(59.6±11.2)%vs.(29.1±12.9)%;FS:(29.9±6.2)%,(31.9±6.2)%vs.(15.2±8.3)%;P均<0.05]。治疗12个月后,治疗组死亡率明显低于对照组(4.8%vs.16.0%,P<0.05)。研究过程中无严重不良事件发生。结论使用卡维地洛治疗EFE,能改善心功能,降低死亡率,改善预后。初始治疗时心功能分级是影响疗效的主要因素。

Objective To analyse the therapeutic effect and influencing factors of carvedilol on primary endocardial fibroelastosis. Methods From January 1990 to December 2012, 46 patients（male 22, female 24） with primary endocardial fibroelastosis（EFE） in the Children＆#39;s Hospital affiliated to Capital Institute of Pediatrics were randomly divided into 2 groups： the control group（n=25） and the treatment group（n=21）. The control group was treated including adrenal cortical hormone, digitalis and diuretic, and treatment group with carvedilol on the basis of the above treatment. Evaluating the left ventricular size, cardiac function, mortality and adverse reactions at before treatment, after treatment of 6 months and 12 months, respectively. Results 5 patients died during the experiment, 4 in the treatment group and 1 in the control group. After 12 months in the treatment group, left ventricular end diastolic diameter significantly decreased[（41.7±6.5） mm vs. （47.0±6.7） mm; P＆lt;0.05]. After 6 months, 12 months in the treatment group, compared with before treatment, left ventricular ejection fraction （EF） and fractional shortening（FS） were significantly increased[EF：（55.6±13.2）%,（59.6±11.2）%vs. （29.1±12.9）%;FS：（29.9±6.2）%, （31.9±6.2）%vs. （15.2±8.3）%;all P＆lt;0.05]. After 12 months of treatment, the mortality was significantly lower in treatment group than that of in the control group（4.8% vs. 16%, P＆lt;0.05）. No serious adverse events occurred during the study. Conclusions Carvedilol can improve cardiac function and the prognosis of primary endocardial fibroelastosis. It is the main influencing factor that the cardiac function classification at the beginning of treatment.