Fontaliran F, Fontaine G, Fillette F, Aouate P, Chomette G, Grosgogeat Y
Laboratoire central d'anatomie pathologique et laboratoire d'électrophysiologie cardiaque, CHE Pitié-Salpêtrière, Paris.
Arch Mal Coeur Vaiss. 1991 Jan;84(1):33-8.
The increasing number of arrhythmogenic idiopathic cardiomyopathies with available histological studies by biopsy or peroperative sampling has prompted the need for data about the normal structure of the right ventricle. A retrospective study of right ventricular tissues was undertaken in 148 autopsies. The quantity of adipose tissue was scored 0 (only epicardial fat visible) to 4 (total replacement of the myocardium by adipose tissue). The topography of the adipocytes was also coded 0 to 4 according to the degree of intermyocytic penetration, irrespective of the total quantify of fat. The amount and extension of adipose tissue in the right ventricular wall was striking. Fifty called "normal" appearances with a myocytic wall of normal thickness and a separate layer of epicardial fat, were only present in 61 of the 148 cases. Adipocytes were present in variable numbers, often unexpectedly high in the majority of cases, with no relationship to the age or gender of the patient with frequent individualization of the myocardial bundles. These surprising findings in normal right ventricles should be born in mind when considering the diagnosis of arrhythmogenic right ventricular dysplasia (ARVD). The histopathological appearances of ARVD are very similar but also differ very significantly, at least in typical cases. The adipocytes are often present in large numbers but this is banal and non-specific. On the other hand, the bundles which penetrate the epicardial fat are usually fibro-muscular and not purely myocytic. In addition, arterioles suggesting segmental diversions are present within the fatty layer with non-specific inflammatory cells.(ABSTRACT TRUNCATED AT 250 WORDS)
通过活检或手术中取样进行组织学研究的致心律失常性特发性心肌病病例越来越多,这就需要了解右心室正常结构的数据。对148例尸检的右心室组织进行了回顾性研究。脂肪组织的数量按0分(仅可见心外膜脂肪)至4分(脂肪组织完全取代心肌)进行评分。脂肪细胞的分布情况也根据肌细胞间的浸润程度编码为0至4分,而不考虑脂肪的总量。右心室壁中脂肪组织的数量和范围令人瞩目。在148例病例中,仅有61例呈现出50种所谓的“正常”外观,即肌细胞壁厚正常且有一层独立的心外膜脂肪。脂肪细胞数量各异,在大多数病例中常常出乎意料地多,与患者的年龄或性别无关,且心肌束常有个体化差异。在考虑致心律失常性右心室发育不良(ARVD)的诊断时,应牢记正常右心室中的这些惊人发现。ARVD的组织病理学表现非常相似,但至少在典型病例中也存在显著差异。脂肪细胞通常数量众多,但这很常见且不具特异性。另一方面,穿透心外膜脂肪的束通常是纤维肌性的,而非纯粹的肌性。此外,在脂肪层内存在提示节段性分流的小动脉,并伴有非特异性炎症细胞。(摘要截断于250字)