Fornes P, Ratel S, Lecomte D
Institute of Forensic Medicine of Paris and Department of Forensic Sciences, College of Medicine Cochin Port-Royal, University of Paris, France.
J Forensic Sci. 1998 Jul;43(4):777-83.
Arrhythmogenic right ventricular dysplasia/ cardiomyopathy (ARVC) is characterized histologically by massive infiltration of the right ventricular wall by fat tissue, with surviving strands of cardiomyocytes bordered by or embedded in fibrosis. ARVC has been recognized as a cause of sudden death, especially in the young. The purpose of our autopsy study was to examine the clinical characteristics and the pathological patterns in the hearts of 20 people who died suddenly of ARVC. In view of our findings and the literature, we discussed the possible causes and pathogenesis of ARVC, as well as the mechanisms by which sudden death occurs in this disease. During the 7-year study period, 20 hearts from 9 men and 11 women fulfilled the criteria for ARVC. The mean age was 41 years (range, 17 to 80). The disease was unknown prior to death in all cases. Fourteen persons died at rest, and six on effort. In 9 of the 20 cases, the trigger of sudden death was an acute emotional stress, sometimes associated with a moderate physical activity. The mean heart weight was 380 g (range, 280 to 520). Both ventricles were involved in 40% of the cases. Inflammatory infiltrates consisting of lymphocytes were present in 60% of the cases, but myocyte necrosis was found in only one case. ARVC is more likely to result from a degenerative process than a congenital disorder. Genetic factors, viral or autoimmune inflammation or both, and apoptosis are also involved in the degenerative disorder.
致心律失常性右室发育不良/心肌病（ARVC）的组织学特征是脂肪组织大量浸润右心室壁，存活的心肌细胞束被纤维组织包绕或嵌入其中。ARVC已被确认为猝死的原因之一，尤其是在年轻人中。我们尸检研究的目的是检查20例因ARVC猝死患者心脏的临床特征和病理模式。鉴于我们的研究结果和相关文献，我们讨论了ARVC可能的病因和发病机制，以及该疾病发生猝死的机制。在7年的研究期间，9名男性和11名女性的20颗心脏符合ARVC标准。平均年龄为41岁（范围17至80岁）。所有病例在死亡前均未被诊断出患有该病。14人死于静息状态，6人死于运动中。20例中有9例，猝死的诱因是急性情绪应激，有时与适度的体力活动有关。平均心脏重量为380克（范围280至520克）。40%的病例双心室均受累。60%的病例存在由淋巴细胞组成的炎性浸润，但仅1例发现心肌细胞坏死。ARVC更可能是由退行性病变而非先天性疾病引起。遗传因素、病毒或自身免疫性炎症或两者兼而有之以及细胞凋亡也与这种退行性疾病有关。
