Chen Xinshan, Zhang Yigu, Rao Guangxun, Huang Guangzhao
Department of Forensic Pathology, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China,
Front Med China. 2007 Jul;1(3):338-42. doi: 10.1007/s11684-007-0065-8. Epub 2007 Jul 1.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of primary myocardial disease characterized by the regional or global replacement of right ventricular myocardium by fatty and fibrolipomatous tissues. The ARVC, usually presenting with different clinical manifestations and pathological changes, were mainly seen in young men and is one of the main causes of sudden death in the young. Here two autopsied cases of Chinese men aged 30 and 23 years old who appeared healthy but died suddenly while at work are reported respectively. One of the victims had extensive and severe pathological changes in his heart involving the left ventricular wall as well as the ventricular septum and the right atrium. Not only was there a global fatty and fibrolipomatous tissue replacement of the right ventricular myocardia, but also mild sarcoplasmic coagulation in the myocardium and focal lymphocytic infiltration in the myocardial interstitium of the right ventricular wall. In addition, slight atherosclerosis of the coronary artery and intimal thickening of the sino-atrial node were observed. It is believed that there are no marked differences in the pathological changes of ARVC between Chinese patients and patients from western countries. The etiology and pathogenesis of ARVC could not be explained by a single cause or factor and they are probably related to various congenital and acquired causes or factors.
致心律失常性右室心肌病(ARVC)是一种原发性心肌病,其特征是右心室心肌被脂肪和纤维脂肪组织区域性或全身性替代。ARVC通常表现出不同的临床表现和病理变化,主要见于年轻男性,是年轻人猝死的主要原因之一。本文分别报告了两名中国男性尸检病例,年龄分别为30岁和23岁,生前看似健康,但在工作时突然死亡。其中一名受害者心脏有广泛而严重的病理变化,累及左心室壁、室间隔和右心房。不仅右心室心肌被脂肪和纤维脂肪组织全身性替代,而且心肌出现轻度肌浆凝聚,右心室壁心肌间质有局灶性淋巴细胞浸润。此外,还观察到冠状动脉轻度动脉粥样硬化和窦房结内膜增厚。据信,中国患者与西方国家患者ARVC的病理变化无明显差异。ARVC的病因和发病机制不能用单一原因或因素来解释,它们可能与各种先天性和后天性原因或因素有关。