Becker A E
Department of Pathology, University of Amsterdam, The Netherlands.
Wien Klin Wochenschr. 1988 Dec 16;100(24):787-91.
A distinction is made between heart muscle diseases with a known cause, or occurring as part of a systemic disease, and those of unknown cause. The first category is termed "specific heart muscle disease". The term "cardiomyopathy" is then limited to the latter category. Cardiomyopathies are categorized as dilated cardiomyopathy, hypertrophic cardiomyopathy and restrictive cardiomyopathy. The pathology of dilated cardiomyopathy depends on the clinical stage of the disease. Basically, the histology shows hypertrophic myocytes, often with degenerative signs, and interstitial fibrosis. Lymphocytes may be observed, particularly in cases studied shortly after the onset of symptoms. Endocardial thickening may occur in time. The role of endomyocardial biopsies in the clinical setting is limited and relates mainly to excluding other diseases, such as myocarditis. The pathology of hypertrophic cardiomyopathy is characterized by myocardial wall thickening, either asymmetric or symmetric, and disorganization of the normal myocardial texture. The latter phenomenon should be distinguished from disarray, since it may occur as a natural phenomenon. The differentiation between these two may be extremely difficult on the basis of only small tissue samples, as with endomyocardial biopsies. This, therefore, poses serious limitations in the use of endomyocardial biopsies. The pathology of restrictive cardiomyopathy also depends on the stage of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
已知病因或作为全身性疾病一部分出现的心肌疾病与病因不明的心肌疾病有所区别。第一类被称为“特异性心肌疾病”。“心肌病”一词则仅限于后者。心肌病分为扩张型心肌病、肥厚型心肌病和限制型心肌病。扩张型心肌病的病理取决于疾病的临床阶段。基本上,组织学显示心肌细胞肥大,常伴有退行性改变及间质纤维化。可能观察到淋巴细胞,尤其是在症状出现后不久进行研究的病例中。心内膜增厚可能随时间出现。心内膜活检在临床环境中的作用有限,主要用于排除其他疾病,如心肌炎。肥厚型心肌病的病理特征为心肌壁增厚,可为不对称或对称增厚,且正常心肌纹理紊乱。后一种现象应与排列紊乱相区分,因为它可能是一种自然现象。仅根据小组织样本(如心内膜活检)很难区分这两者。因此,这对心内膜活检的应用造成了严重限制。限制型心肌病的病理也取决于疾病阶段。(摘要截断于250字)