title: Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria.
abstract: 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.
  
  
  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 modification of the Task Force criteria, quantitative criteria are proposed and abnormalities are defined on the basis of comparison with normal subject data.
  
  
  CONCLUSIONS
  The present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition. Clinical Trial Registration clinicaltrials.gov Identifier: NCT00024505.
authors.affiliation: University of Arizona, Tucson, AZ, USA. fmarcus@u.arizona.edu
authors.forename: Frank I
authors.initials: FI
authors.lastname: Marcus
authors.forename: William J
authors.initials: WJ
authors.lastname: McKenna
authors.forename: Duane
authors.initials: D
authors.lastname: Sherrill
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.forename: Barbara
authors.initials: B
authors.lastname: Bauce
authors.forename: David A
authors.initials: DA
authors.lastname: Bluemke
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
authors.forename: Domenico
authors.initials: D
authors.lastname: Corrado
authors.forename: Moniek G P J
authors.initials: MG
authors.lastname: Cox
authors.forename: James P
authors.initials: JP
authors.lastname: Daubert
authors.forename: Guy
authors.initials: G
authors.lastname: Fontaine
authors.forename: Kathleen
authors.initials: K
authors.lastname: Gear
authors.forename: Richard
authors.initials: R
authors.lastname: Hauer
authors.forename: Andrea
authors.initials: A
authors.lastname: Nava
authors.forename: Michael H
authors.initials: MH
authors.lastname: Picard
authors.forename: Nikos
authors.initials: N
authors.lastname: Protonotarios
authors.forename: Jeffrey E
authors.initials: JE
authors.lastname: Saffitz
authors.forename: Danita M Yoerger
authors.initials: DM
authors.lastname: Sanborn
authors.forename: Jonathan S
authors.initials: JS
authors.lastname: Steinberg
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.forename: Adalena
authors.initials: A
authors.lastname: Tsatsopoulou
authors.forename: Thomas
authors.initials: T
authors.lastname: Wichter
authors.forename: Wojciech
authors.initials: W
authors.lastname: Zareba
mesh_terms: D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D001706:Biopsy; D004562:Electrocardiography; D005838:Genotype; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000592:standards; D009206:Myocardium / Q000473:pathology; D010641:Phenotype; D017410:Practice Guidelines as Topic; D012680:Sensitivity and Specificity