Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia.
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DOI:
10.1016/j.amjcard.2009.11.033
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发表时间:
2010-04-01
影响因子:
2.8
通讯作者:
Sorrell, Vincent L.
Sorrell, Vincent L.
中科院分区:
医学3区
文献类型:
--
作者:
Marcus, Frank;Basso, Cristina;Gear, Kathleen;Sorrell, Vincent L.

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致心律失常性右心室心肌病/发育不良(ARVC/D)的诊断基于1994年发布的特别工作组标准,其中包括右心室成像异常以及通过心内膜心肌活检对右心室心肌进行诊断病理评估。这些最近已被修改,包括心脏磁共振(CMR)的评价。此外,新标准还包括纤维化百分比和肌细胞减少的定量标准。在尸检时确定是否存在ARVC/D的缺陷以及评估家庭成员是否存在这种疾病的困难在以下报告中得到了很好的说明。总之,本报告说明了需要订阅修改后的标准,以避免误诊。
The diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is based on Task Force Criteria published in 1994 that included imaging abnormalities of the right ventricle as well as diagnostic pathological evaluation of the right ventricular myocardium by endomyocardial biopsy. These have recently been modified to include evaluation by cardiac magnetic resonance (CMR). In addition quantitative criteria for the percent of fibrosis and decrease in myocyte are included in the new criteria. The pitfalls of determining the presence of ARVC/D at autopsy and the difficulty of assessing the presence of this disease in family members are well illustrated in the following report. In conclusion, this report illustrates the need to subscribe to the modified criteria to avoid misdiagnosis.
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