Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a matter of fat?
Adipositas cordis, fatty infiltration of the right ventricle, and arrhythmogenic right ventricular cardiomyopathy. Just a matter of fat?
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DOI：
10.1016/j.carpath.2004.12.001
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发表时间：
2005-01-01
影响因子：
3.7
通讯作者：
Thiene, G
中科院分区：
文献类型：
--
作者：
Basso, C;Thiene, G
右室脂肪浸润是否必须被认为是致心律失常性右室心肌病(ARVC)的一个充分的形态特征仍是一个争议的来源；ARVC应该与右室脂肪浸润和心脏脂肪组织区分开来。事实上，众所周知，即使在正常心脏中，右室前外侧和心尖区也存在一定量的心肌内脂肪，而且心外膜脂肪随着体重的增加而增加。然而，ARVC的纤维脂肪和脂肪变异除了表现为右室心肌脂肪置换外，还表现为心肌细胞退行性改变和间质纤维化，伴或不伴广泛的替换型纤维化。不仅在临床环境中，而且在法医和普通病理学领域，都有必要采取严格的诊断标准。当处理猝死病例时，唯一的形态学表现是心外膜或心肌内脂肪增多，应始终寻找更令人信服的致心律失常的来源，如心肌炎性浸润物、纤维化、异常通路和离子通道疾病，以避免对ARVC病例的过度诊断。(C)2005 Elsevier Inc.保留所有权利。
Whether fatty infiltration of the right ventricle has to be considered "per se" a sufficient morphologic hallmark of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still a source of controversy; ARVC should be kept distinct from both fatty infiltration of the right ventricle and adipositas cordis. In fact, it is well known that a certain amount of intramyocardial fat is present in the right ventricular anterolateral and apical regions even in the normal heart and that the epicardial fat increases with increasing body weight. However, both the fibro-fatty and fatty variants of ARVC show, besides fatty eplacement of the right ventricular myocardium, degenerative changes of the myocytes and interstitial fibrosis, with or without extensive replacement-type fibrosis. The need to adopt strict diagnostic criteria is warranted not only in the clinical setting but also in the forensic and general pathology arena. When dealing with a case of sudden death, in which the only morphologic finding consists of an increased amount of epicardial or intramyocardial fat, a more convincing arrhythmogenic source such as myocardial inflammatory infiltrates, fibrosis, anomalous pathways, and ion channel disease should always be searched for, in order to avoid an over-diagnosis of ARVC cases. (C) 2005 Elsevier Inc. All rights reserved.