Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.
Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.
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DOI：
10.1161/circulationaha.108.840827
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发表时间：
2010-04-06
期刊：
影响因子：
37.8
通讯作者：
Zareba W
中科院分区：
文献类型：
--
作者：
Marcus FI;McKenna WJ;Sherrill D;Basso C;Bauce B;Bluemke DA;Calkins H;Corrado D;Cox MG;Daubert JP;Fontaine G;Gear K;Hauer R;Nava A;Picard MH;Protonotarios N;Saffitz JE;Sanborn DM;Steinberg JS;Tandri H;Thiene G;Towbin JA;Tsatsopoulou A;Wichter T;Zareba W
1994年，一个国际工作组提出了ARVC/D的临床诊断标准，该标准有助于识别和解释ARVC/D的非特异性临床特征，从而能够通过排除表型来对指征病例进行确证临床诊断，并为临床研究和遗传学研究提供了一个标准。ARVC/D的结构、组织学、心电图、心律失常和家族性特征被纳入标准，并根据其与ARVC/D的相关性的特殊性分为重大和轻微。当时，ARVC/D的临床经验主要是有症状的病例和心脏性猝死患者：明显和/或严重的疾病谱。因此，1994年的标准具有高度的特异性，但对早期和家族性疾病缺乏敏感性。诊断标准的修订为新出现的诊断模式的作用和ARVC/D遗传学方面的进展提供了指导。对标准进行了修改，以纳入新的知识和技术，以提高诊断的敏感性，但重要的是保持诊断的特异性。将疾病的结构、组织学、心电图、心律失常和遗传特征分类为主要和次要标准的方法一直保持不变。在对工作队标准的这次修订中，提出了量化标准，并根据与正常受试者数据的比较确定了异常情况。在修改特别工作组原有标准的基础上诊断ARVC/D是改进这种情况的诊断和管理的工作框架。
In 1994, an International Task Force proposed criteria for the clinical diagnosis of ARVC/D which facilitated recognition and interpretation of the frequently non-specific clinical features of ARVC/D. This enabled confirmatory clinical diagnosis in index cases through exclusion of phenocopies, and provided a standard upon which clinical research and genetic studies could be based. Structural, histological, electrocardiographic, arrhythmic, and familial features of the disease were incorporated into the criteria, subdivided into major and minor according to the specificity of their association with ARVC/D. At that time, clinical experience with ARVC/D was dominated by symptomatic index cases and sudden cardiac death victims: the overt and/or severe end of the disease spectrum. Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease. Revision of the diagnostic criteria provides guidance on the role of emerging diagnostic modalities and advances in the genetics of ARVC/D. The criteria have been modified to incorporate new knowledge and technology to improve diagnostic sensitivity, but with the important requisite of maintaining diagnostic specificity. The approach classifying structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease as major and minor criteria has been maintained. In this modification of the Task Force Criteria, quantitative criteria are proposed and abnormalities are defined based on comparison with normal subject data. The diagnosis of ARVC/D based on modification of the original Task Force criteria is a working framework to improve the diagnosis and management of this condition.