Department of Pathology, Escola Paulista de Medicina, UNIFESP, Sao Paulo 04023-900, Brazil.
Hum Pathol. 2012 Apr;43(4):592-6. doi: 10.1016/j.humpath.2011.06.014. Epub 2011 Sep 19.
Arrhythmogenic cardiomyopathy is a rare cardiomyopathy characterized by fibrofatty replacement primarily of the right ventricular myocardium. It is a major cause of sudden death in the young and in athletes. There are few autopsy studies of the ventricular distribution of the disease. Fifty cases of sudden cardiac death with fibrofatty replacement in either ventricle from a single medical examiner's office were studied. Distribution of disease as determined grossly and microscopically was correlated with activity at time of death, race, and presence of inflammation. Extent of disease was right ventricular in 6 cases (12%; age, 25 ± 5 years), biventricular in 25 (50%; age, 36 ± 3 years), and left ventricular in 19 (38%; age, 37 ± 3 years) (P = .13). Inflammation was present in 44% of biventricular arrhythmogenic cardiomyopathy versus 74% of left ventricular arrhythmogenic cardiomyopathy and 83% of right ventricular arrhythmogenic cardiomyopathy (P = .06). Arrhythmogenic cardiomyopathy, when presenting with sudden death, is usually biventricular. There is a trend that univentricular involvement occurs at an earlier age and that right ventricular involvement shows more inflammation, suggesting different stages of disease.
致心律失常性右室心肌病是一种罕见的心肌病，其特征为纤维脂肪组织主要替代右心室心肌。它是年轻人和运动员猝死的主要原因。关于该病心室分布的尸检研究很少。研究了来自单一法医办公室的 50 例因纤维脂肪替代而导致心源性猝死的病例。大体和显微镜下确定的疾病分布与死亡时的活动、种族和炎症的存在相关。疾病的严重程度在 6 例（12%；年龄 25 ± 5 岁）中为右心室，在 25 例（50%；年龄 36 ± 3 岁）中为双心室，在 19 例（38%；年龄 37 ± 3 岁）中为左心室（P =.13）。炎症存在于 44%的双心室致心律失常性心肌病中，而左心室致心律失常性心肌病中为 74%，右心室致心律失常性心肌病中为 83%（P =.06）。致心律失常性心肌病在发生猝死时通常为双心室受累。有一种趋势表明，单侧心室受累发生在较早的年龄，而右心室受累表现出更多的炎症，这表明疾病处于不同的阶段。
