Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

• DOI: 10.1161/circulationaha.108.840827
• Publication Date: 2010-02-20T01:44:27Z
• Authors: Frank I. Marcus; William J. McKenna; Duane Sherrill; Cristina Basso; Barbara Bauce; David A. Bluemke; Hugh Calkins; Domenico Corrado; Moniek G.P.J. Cox; James P. Daubert; Guy Fontaine; Kathleen Gear; Richard Hauer; Andrea Nava; Michael H. Picard; Nikos Protonotarios; Jeffrey E. Saffitz; Danita M. Yoerger...; Jonathan S. Stein...; Harikrishna Tandri; Gaetano Thiene; Jeffrey A. Towbin; Adalena Tsatsopoulou; Thomas Wichter; Wojciech Zareba
• Keywords: Sudden Death; Phenocopy

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 frequently nonspecific features ARVC/D. This enabled confirmatory in index cases through exclusion phenocopies provided a standard on which research genetic studies could be based. Structural, histological, electrocardiographic, arrhythmic, familial disease were incorporated into criteria, subdivided major minor categories according to specificity their association with At time, experience ARVC/D was dominated by symptomatic sudden cardiac death victims—the overt or severe end spectrum. Consequently, 1994 highly specific but lacked sensitivity early disease. Methods Results— Revision diagnostic provides guidance role emerging modalities advances genetics The have been modified incorporate new knowledge technology improve sensitivity, important requisite maintaining specificity. approach classifying structural, as has maintained. this modification quantitative are abnormalities defined basis comparison normal subject data. Conclusions— present modifications Criteria represent working framework management condition. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00024505.