Guiraudon C M
Department of Pathology, University Hospital, University of Western Ontario, London, Canada.
Eur Heart J. 1989 Sep;10 Suppl D:95-6. doi: 10.1093/eurheartj/10.suppl_d.95.
Right ventricular dysplasia is a rare cardiomyopathy predominantly involving the right ventricle. We report eight cases studied with light and electron microscopy. By light microscopy, we found the typical replacement of myocardium with mature adipose tissue where only scattered degenerated myocytes wrapped in thin fibrous sheets are seen. Electron microscopy shows abnormal intercalated discs in both the right and left ventricles: intercalated discs are attenuated and flattened with decreased Z band material (desmin). These changes, not previously reported, may be useful for diagnosing ARVD.
右心室发育不良是一种罕见的心肌病,主要累及右心室。我们报告了8例经光镜和电镜研究的病例。通过光镜,我们发现心肌被成熟脂肪组织典型替代,仅可见散在的包裹在薄纤维片中的退变心肌细胞。电镜显示右心室和左心室的闰盘均异常:闰盘变薄且扁平,Z带物质(结蛋白)减少。这些此前未被报道的变化可能有助于诊断致心律失常性右室心肌病(ARVD)。