TY - JOUR
T1 - Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia
AU - Marcus, Frank I.
AU - McKenna, William J.
AU - Sherrill, Duane
AU - Basso, Cristina
AU - Bauce, Barbara
AU - Bluemke, David A.
AU - Calkins, Hugh
AU - Corrado, Domenico
AU - Cox, Moniek G.P.J.
AU - Daubert, James P.
AU - Fontaine, Guy
AU - Gear, Kathleen
AU - Hauer, Richard
AU - Nava, Andrea
AU - Picard, Michael H.
AU - Protonotarios, Nikos
AU - Saffitz, Jeffrey E.
AU - Sanborn, Danita M.Yoerger
AU - Steinberg, Jonathan S.
AU - Tandri, Harikrishna
AU - Thiene, Gaetano
AU - Towbin, Jeffrey A.
AU - Tsatsopoulou, Adalena
AU - Wichter, Thomas
AU - Zareba, Wojciech
N1 - Funding Information:
This project was supported by National Institutes of Health research grant R13 HL086825, funded by the National Heart, Lung & Blood Institute and the Office of Rare Disorders, and was supported in part by research grants U01-HL65594, U01-HL65652, U01-HL65691, and K23-HL093350 from the National Heart, Lung and Blood Institute of the National Institutes of Health, Bethesda, MD, and by research grant QLG1-CT-2000-01091 5th Framework Programme from the European Commission, Brussels, Belgium. Additional funding was obtained from the International Society for Holter and Noninvasive Electrocardiography and donations from the Peter French Memorial Foundation, United Desert Charities, the Podolsky Family Foundation, and private donors Mr and Mrs L. Becker, Mr and Mrs H. Danz, Mr K. Dorn, Mr T. Livolsi, Mr and Mrs L. Long, and Mr and Mrs H. Wilmerding.
PY - 2010/4
Y1 - 2010/4
N2 - BackgroundIn 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 ResultsRevision 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.ConclusionsThe present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition.Clinical Trial Registrationclinicaltrials.gov Identifier: NCT00024505.
AB - BackgroundIn 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 ResultsRevision 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.ConclusionsThe present modifications of the Task Force Criteria represent a working framework to improve the diagnosis and management of this condition.Clinical Trial Registrationclinicaltrials.gov Identifier: NCT00024505.
KW - Arrhythmias, cardiac
KW - Arrhythmogenic right ventricular cardiomyopathy/ dysplasia
KW - Death, sudden, cardiac
KW - Diagnosis
KW - Echocardiography
KW - Electrocardiography
KW - Magnetic resonance imaging
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U2 - 10.1093/eurheartj/ehq025
DO - 10.1093/eurheartj/ehq025
M3 - Article
C2 - 20172912
AN - SCOPUS:77950482741
VL - 31
SP - 806
EP - 814
JO - European Heart Journal
JF - European Heart Journal
SN - 0195-668X
IS - 7
ER -