Pathologic Anatomy of the Cardiomyopathies
Pathologic Anatomy of the Cardiomyopathies
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心肌病的病理解剖学
DOI：
10.1016/s0046-8177(75)90003-9
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发表时间：
1975
期刊：
影响因子：
3.3
通讯作者：
V. Ferrans
中科院分区：
文献类型：
--
作者：
W. Roberts;V. Ferrans
本报告总结了三种类型心肌病患者的尸检观察结果：特发性、浸润性和肌内膜炎性疾病。根据心室腔的大小，特发性类型可分为两种类型。在扩张心室型中，左心室壁常常小于1.5cm。心内厚血栓常见，房室瓣环通常轻度扩张，局灶性心肌和内膜瘢痕常见。在非扩张型（肥厚型心肌病）中，室间隔通常比左心室游离壁厚，后者也厚（大于1.5 cm）。当室间隔厚度与左心室游离壁厚度相似（对称）时，不会发生左心室流出道梗阻。当隔膜比左心室自由壁厚（不对称）时，可能存在或可能不存在左心室或右心室流出道阻塞。非扩张型（肥厚型）室间隔内心肌纤维相互间的定向异常，而扩张型室间隔内心肌纤维相互间的定向正常。非扩张型的特发性心脏肥大，心内血栓罕见，房室瓣环从不扩张。心肌病的浸润类型包括铁、钙、脂质、粘多糖、肉芽肿、淀粉样蛋白和肿瘤。前四个通常位于心肌细胞内，后三个位于心肌细胞之间。很可能所有这些心肌浸润都能够产生心功能障碍，主要是在限制性基础上。肌内膜炎可能与嗜酸性粒细胞增多症有关，也可能无关。当后者发生时，嗜酸性粒细胞结构正常。死亡与充血性心力衰竭有关。这一类别在特发性疾病中也是现实的。
This presentation summarizes necropsy observations in patients with three types of cardiomyopathy: idiopathic, infiltrative, and endomyocardial disease. The idiopathic variety is subdivided into two types depending on the size of the ventricular cavity. In the dilated ventricular type the left ventricular wall is frequently less than 1.5 cm. thick, intracardiac thrombi are common, the atrioventricular valve rings usually are mildly dilated, and focal myocardial and endocardial scars are common. In the nondilated type (hypertrophic cardiomyopathy), the ventricular septum is usually thicker than the left ventricular free wall, which also is thick (greater than 1.5 cm.). When the septum is similar in thickness to the left ventricular free wall (symmetric), left ventricular outflow obstruction does not occur. When the septum is thicker than the left ventricular free wall (asymmetric), left or right ventricular outflow obstruction may or may not be present. The orientation of myocardial fibers one to another in the ventricular septum in the nondilated (hypertrophic) type is abnormal, whereas it is normal in the dilated ventricular type. Intracardiac thrombi are rare and atrioventricular valve rings are never dilated in the nondilated type of idiopathic cardiomegaly. The infiltrative types of cardiomyopathies include iron, calcium, lipids, mucopolysaccharides, granulomas, amyloid, and neoplasms. The first four usually are located within myocardial cells and the latter three, between myocardial cells. It is probable that all these myocardial infiltrates are capable of producing cardiac dysfunction, primarily on a restrictive basis. Endomyocardial disease may or may not be associated with eosinophilia. When the latter occurs, the eosinophils are structurally normal. Death is related to congestive cardiac failure. This category is actuality also in idiopathic.