Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia

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 Sanborn, Jonathan S. Steinberg, Harikrishna Tandri & 5 others Gaetano Thiene, Jeffrey Towbin, Adalena Tsatsopoulou, Thomas Wichter, Wojciech Zareba

Research output: Contribution to journalArticle

628 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)806-814
Number of pages9
JournalEuropean Heart Journal
Volume31
Issue number7
DOIs
StatePublished - Jan 1 2010
Externally publishedYes

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Arrhythmogenic Right Ventricular Dysplasia
Advisory Committees
Genetic Research
Inborn Genetic Diseases
Sudden Cardiac Death
Clinical Trials
Technology

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Marcus, F. I., McKenna, W. J., Sherrill, D., Basso, C., Bauce, B., Bluemke, D. A., ... Zareba, W. (2010). Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. European Heart Journal, 31(7), 806-814. https://doi.org/10.1093/eurheartj/ehq025

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. / Marcus, Frank I.; McKenna, William J.; Sherrill, Duane; Basso, Cristina; Bauce, Barbara; Bluemke, David A.; Calkins, Hugh; Corrado, Domenico; Cox, Moniek G.P.J.; Daubert, James P.; Fontaine, Guy; Gear, Kathleen; Hauer, Richard; Nava, Andrea; Picard, Michael H.; Protonotarios, Nikos; Saffitz, Jeffrey E.; Sanborn, Danita M.Yoerger; Steinberg, Jonathan S.; Tandri, Harikrishna; Thiene, Gaetano; Towbin, Jeffrey; Tsatsopoulou, Adalena; Wichter, Thomas; Zareba, Wojciech.

In: European Heart Journal, Vol. 31, No. 7, 01.01.2010, p. 806-814.

Research output: Contribution to journalArticle

Marcus, FI, McKenna, WJ, Sherrill, D, Basso, C, Bauce, B, Bluemke, DA, Calkins, H, Corrado, D, Cox, MGPJ, Daubert, JP, Fontaine, G, Gear, K, Hauer, R, Nava, A, Picard, MH, Protonotarios, N, Saffitz, JE, Sanborn, DMY, Steinberg, JS, Tandri, H, Thiene, G, Towbin, J, Tsatsopoulou, A, Wichter, T & Zareba, W 2010, 'Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia', European Heart Journal, vol. 31, no. 7, pp. 806-814. https://doi.org/10.1093/eurheartj/ehq025
Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. European Heart Journal. 2010 Jan 1;31(7):806-814. https://doi.org/10.1093/eurheartj/ehq025
Marcus, Frank I. ; McKenna, William J. ; Sherrill, Duane ; Basso, Cristina ; Bauce, Barbara ; Bluemke, David A. ; Calkins, Hugh ; Corrado, Domenico ; Cox, Moniek G.P.J. ; Daubert, James P. ; Fontaine, Guy ; Gear, Kathleen ; Hauer, Richard ; Nava, Andrea ; Picard, Michael H. ; Protonotarios, Nikos ; Saffitz, Jeffrey E. ; Sanborn, Danita M.Yoerger ; Steinberg, Jonathan S. ; Tandri, Harikrishna ; Thiene, Gaetano ; Towbin, Jeffrey ; Tsatsopoulou, Adalena ; Wichter, Thomas ; Zareba, Wojciech. / Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. In: European Heart Journal. 2010 ; Vol. 31, No. 7. pp. 806-814.
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abstract = "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.",
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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

AU - Tsatsopoulou, Adalena

AU - Wichter, Thomas

AU - Zareba, Wojciech

PY - 2010/1/1

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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.

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