心内膜弹力纤维增生症临床病理特征及误诊原因分析

Clinical pathological features of endocardial fibroelastosis and analysis the reasons of misdiagnosis

目的进一步认识心内膜弹力纤维增生症的临床病理学特征,分析误诊的原因。方法收集3例学龄前儿童尸检病例,结合临床病史、心电图、心脏超声心动图检查及病理学常规、特殊染色,并复习相关文献。结果眼观:心脏增大,质量增加,以左心室增大为主;心内膜呈弥漫或不规则增厚达0.3～0.5cm。镜检:心内膜弹力纤维及胶原纤维增生,平行排列,弹力纤维染色阳性。结论对于突发心力衰竭的患儿,临床医师应重视X线、心电图及超声心动图检查,必要时做心内膜活检确诊。

Purpose To investigate the clinicpathological features of endocardial fibroelastosis （EFE） and analysis the reasons of misdiagnosis. Methods Three autopsy cases of preschool children was collected. The clinical data included electrocardiogram, echocardiography, histopathology and special stain. Related literatures were reviewed. Results All of 3 cases had cordis morphological changes, dominating changes were augmentation of left ventricle. It was found the diffused or anomalistic incrassation of endocardium. The main histopathologic feature was the endocardial thickening due to proliferation of elastin and collagen. Histochemical staining of elastin was positive. Conclusions It is suggested that clinician should think highly of X-rays, electrocardiogram and echocardiography for suddenly heart failure in infancy. It is necessary to do the endomyocardial biopsy.