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目的:探讨终末期心脏病的心肌组织病理改变及特征。方法:对接受心脏移植的9例扩张型心肌病(DCM)患者、6例其他类型终末期心脏病患者[肥厚型心肌病(HCM)2例,缺血性心肌病、心脏横纹肌肉瘤、先天性心脏病房间隔缺损、法洛四联症各1例]及1例正常对照的心肌标本进行组织病理学和电镜检查,并与临床心功能分级对照分析。结果:DCM患者光镜下均可见广泛纤维组织增生,以血管周围最为明显,心功能Ⅲ~Ⅳ级的心肌纤维化程度为,较心功能Ⅱ级者(+)为重;另可见心肌细胞形态异常,但少见间质炎症细胞浸润、心肌细胞排列紊乱及小血管变化;电镜下均可见肌原纤维稀疏,线粒体肿胀、形态不规则,其中呈线粒体空泡样变、巨大线粒体、肌质网明显扩张各1例。1例HCM患者光镜下呈现与DCM者类似的改变,但肌原纤维有断裂现象,细胞排列紊乱,胞核畸形,血管扩张,较多炎症细胞浸润;1例HCM患者及2例终末期先天性心脏病患者的心肌组织病理学改变与DCM患者相似。6例其他类型终末期心脏病患者电镜下的心肌超微结构改变均与DCM患者相似,但未见有巨大线粒体。结论:①心肌纤维化是终末期心脏病心肌组织病理学的主要特征,纤维化程度越重,心功能越差;②心肌细胞排列紊乱及炎症细胞浸润可能为HCM的特征性改变;③线粒体异常如巨大线粒体可能为终末期DCM的特征性改变。
Objective: To investigate the pathological changes and characteristics of myocardial tissue in patients with end-stage heart disease. METHODS: Nine patients with dilated cardiomyopathy (DCM) undergoing heart transplantation and six patients with other types of end-stage heart disease (hypertrophic cardiomyopathy (HCM), ischemic cardiomyopathy, rhabdomyosarcoma of the heart, congenital Heart disease atrial septal defect, tetralogy of Fallot each case] and 1 normal control myocardial samples histopathological and electron microscopic examination, and clinical cardiac function grading control analysis. Results: In DCM patients, extensive fibrous tissue hyperplasia was observed under the light microscope, with the most obvious perivascular findings. The degree of myocardial fibrosis of grade Ⅲ ~ Ⅳ was higher than that of grade Ⅱ (+), and the shape of myocardial cells Abnormal, but rare interstitial inflammatory cell infiltration, myocardial cell dysfunction and small changes in blood vessels; under electron microscopy can be seen myofibril sparse, mitochondria swelling, irregular shape, which showed mitochondrial vacuolar degeneration, huge mitochondria, sarcoplasmic reticulum 1 case of expansion. One case of HCM showed similar changes in light microscope and DCM, but myofibrils were broken, the cells arranged in disorder, nuclear deformity, vasodilation and infiltration of more inflammatory cells. One case of HCM and two cases of end-stage congenital Myocardial histopathological changes in patients with cardiac disease are similar to DCM patients. Electrocardiographic ultrastructural changes in 6 patients with other types of end-stage heart disease were similar to DCM patients, but no significant mitochondria were seen. Conclusion: (1) Myocardial fibrosis is the main characteristic of myocardial histopathology in patients with end-stage heart disease. The more serious the degree of fibrosis is, the worse the cardiac function. (2) The imbalance of myocardial cells and infiltration of inflammatory cells may be characteristic changes of HCM. (3) Such as the huge mitochondria may be the characteristic changes of end-stage DCM.