小儿原发性心肌病致心力衰竭的临床诊治分析

Clinical analysis of diagnose and treatment in heart failure due to pediatric primary cardiomyopathy

目的分析小儿原发性心肌病心力衰竭的最新诊治进展,探讨左心室辅助装置(leftventricular assist device,LVAD)的作用机制及其在小儿原发性心肌病心力衰竭中的应用价值。方法1995年3月至2006年3月收治的44例原发性心肌病心衰患儿,其中3例内科药物治疗无效及病情进展迅速的患儿采用手术安装LVAD代替左心室行使泵功能。分析DCM组和EFE组中相关指标:肝大,气急,心胸比率(CTR),射血分数(EF),短轴缩短率(FS)。结果两组病例中EF均值分别为(39.00±11.90)%和(48.00±10.80)%,FS为(16.57±5.59)%和(20.00±5.97)%。3例DCM患儿于Innsbruck医科大学附属儿童医院接受手术安装LVAD。结论在对小儿心力衰竭的诊断中,应强调客观检查指标EF和FS等的重要性及主观检测项目的标准化。治疗上β-受体阻滞剂的配合应用有一定相辅相成的效果,值得进一步推广。而目前国际上LVAD如Berlin Heart系统的应用,在控制心衰上取得良好的效果,但因各种因素的限制,目前在国内儿科界开展尚需努力。

Objective To observe diagnostic criteria and treatment of heart failure in pediatric primary cardiomyopathy, improve diagnostic standardization and therapeutic efficiency of pediatric heart failure. Analyse the mechanism of LVAD （left ventricular assist device）. Methods There were 44 cases of heart failure in DCM and EFE from March 1995 to March 2006, analysed and compared with their clinical signs,CTR,EF and FS. The patients in routine group were treated by routine therapy for heart failure （digitalis, diuretics and vasodilator） and 10 patients were treated byβ-blocker on the basic of routine therapy. The therapeutic effects in the two groups were observed. LVAD（Berlin Heart System） was undertaken to replace the pump function of ventricle in these patients who had been unresponsive to drugs and developed severe heart failure. Results In 25 children in DCM group with heart failure, the average of CTR is （68±5.9）%, with hepatomegaly 19 cases （19/25）,with tachypnea 21 cases（21/25）. In 19 children in EFE group with heart failure, the average of CTR is 67± 6. 02%, with hepatomegaly 9 cases（9/19）,with tachypnea 16 cases（16/19）. The average of EF in DCM group is 39±11.9%, FS is 16. 57±5.59%. In EFE group, EF is 48± 10.8%, FSis 20± 5.97. After treatment by routine therapy for heart failure, Cardiac function were improved in most of patients, but 2 patients died. 10 patients were treated by routine therapy plus β-blocker, all have improving cardiac function till now. In medical university of Innsburck, LVAD was utilized in a 6 months boy. LVAD first in a 40 months boy, then changed to BIVD for the right heart failure. BIVD in a 13 years girl. Conclusion The important role of EF and FS in children patients with heart failure should be focused on. At the same time, emphasizing on standardization in methods of subjective examination. The therapeutic effect of routine therapy plusβ- blocker is much better than that of simple routine therapy. As a new technique in the treatment of children heart failure patients, LVAD has achieved good effects in the therapy of primary pediatric cardiomyopathy cases and is worth further effort in pediatric field.