心内膜弹力纤维增生症研究进展

Advance in research on endocardial fiborelastosis

心内膜弹力纤维增生症(EFE)是一种常见的婴儿心肌病,其发病可能与病毒感染、遗传、免疫、心内膜发育不良等因素有关。心内膜弹力纤维及胶原纤维增生是EFE基本病理改变。EFE的临床分型有多种,以急性型多见。其临床表现缺乏特异性,充血性心力衰竭是主要临床表现。超声心动图在诊断中具有重要意义。应注意与肺炎并发心衰、病毒性心肌炎、左冠状动脉起源异常等疾病相鉴别。治疗上主要控制心力衰竭,对洋地黄治疗反应良好而又能长期坚持吃药者,预后较好。

Endocardial fiborelastosis （EFE） is a common infantile myocardiosis. The pathogenesis of EFE may be associated with viral infection, genetic factors, immune factors and endocardial dysplasia. The fundamental pathological changes of EFE include hyperplasia of endocardium elastic fibers and collagen fibers. Acute EFE is a frequent type. Clinical manifestations of EFE are non-specific and children with EFE mainly present with congestive heart failure. Echocardiography is very helpful to the diagnosis of EFE. It is necessary to differentiate EFE from pneumonia complicated by acute congestive heart failure, viral myocarditis and anomalous origin of the left coronary artery. Treatment is meant to control symptoms of congestive heart failure. Patients who respond well to digitalis and have good medication compliance have a favorable prognosis.