Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

作者：Gregory M. Marcus, William John McKenna, Duane L. Sherrill, Cristina Basso, Barbara Bauce, David A. Bluemke, Hugh G Calkins, Domenico Corrado, Moniek G.P.J. Cox, James P. Daubert, Guy Hugues Fontaine, Kathleen Gear, Richard N.W. Hauer, Andrea Kazemi Nava, Michael H. Picard, Nikos Protonotarios, Jeffrey E. Saffitz, Danita M. Yoerger Sanborn, Jonathan S. Steinberg, Harikrishna S. Tandri, Gaetano Thiene, Jeffrey Allen Towbin, Adalena Tsatsopoulou, Thomas Wichter, Wojciech Zaręba · 发表于：Circulation · 年份：2010 · DOI：10.1161/circulationaha.108.840827 · 被引用次数：2685 · 研究领域：Cardiovascular Effects of Exercise、Cardiac electrophysiology and arrhythmias、Eosinophilic Disorders and Syndromes

BACKGROUND: In 1994, an International Task Force proposed criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and interpretation of the frequently nonspecific clinical features of ARVC/D. This enabled confirmatory clinical diagnosis in index cases through exclusion of phenocopies and provided a standard on 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 categories 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 or severe end of the disease spectrum. Consequently, the 1994 criteria were highly specific but lacked sensitivity for early and familial disease. METHODS AND RESULTS: 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 of classifying structural, histological, electrocardiographic, arrhythmic, and genetic features of the disease as major and minor criteria has been maintained. In this modific...