Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Proposed Modification of the Task Force Criteria
Open accessAbstract
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 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— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00024505.
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ACM is now recognized as a spectrum of cardiomyopathic phenotypes, including right-dominant (ARVC), left-dominant (ALVC), and biventricular forms (ACM).
“Although originally described as a rare inherited heart disease confined to the right ventricle, ACM is now recognized as a spectrum of cardiomyopathic phenotypes, including right-dominant (ARVC), left-dominant (ALVC), and biventricular forms (ACM), reflecting substantial genetic, molecular, and environmental heterogeneity [8,9].”
From A Paradigm Shift: Arrhythmogenic Cardiomyopathy Is an Inflammatory Disease (2026) · cites this paper as [8]
The inability to discern normal nulled RV free wall myocardium in a majority of athletes more likely reflects limitations in spatial resolution of the CMR technique rather than abnormal enhancement.
“While this could be interpreted as abnormal enhancement, it is more likely to reflect the limitations in spatial resolution of the technique, a sentiment echoed by a panel of experts who deemed that DGE assessment in the RV free wall was too unreliable to be included in the recent revision of diagnostic criteria for arrhythmogenic RV cardiomyopathy.35”
From Exercise-induced right ventricular dysfunction and structural remodelling in endurance athletes (2012) · cites this paper as [35]
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- Authors
- Marcus, F. I., McKenna, W. J., Sherrill, D., Basso, C., Bauce, B., Bluemke, D. A., Calkins, H., Corrado, D., Cox, M. G. P. J., Daubert, J. P., Fontaine, G., Gear, K., Hauer, R., Nava, A., Picard, M. H., Protonotarios, N., Saffitz, J. E., Sanborn, D. M. Y., Steinberg, J. S., Tandri, H., Thiene, G., Towbin, J. A., Tsatsopoulou, A., Wichter, T., & Zareba, W.
- Affiliations
- From the University of Arizona (F.I.M., D.S., K.G.), Tucson, Ariz; The Heart Hospital, London, United Kingdom (W.J.M.); University of Padua Medical School, Padua, Italy (C.B., B.B., D.C., A.N., G.T.); National Institutes of Health, Clinical Center (D.A.B.), Bethesda, and Johns Hopkins Hospital (H.C., H.T.), Baltimore, Md; University Medical Center Utrecht, Utrecht, the Netherlands (M.G.P.J.C., R.H.); Strong Memorial Hospital (J.P.D.) and University of Rochester Medical Center (W.Z.), Rochester, NY;...
- University of Arizona (US)
- ORCID
- McKenna, William J.
- Basso, Cristina
- Bauce, Barbara
- Bluemke, David A.
- Calkins, Hugh
- Corrado, Domenico
- Cox, Moniek G.P.J.
- Daubert, James P.
- Fontaine, Guy
- Hauer, Richard
- Nava, Andrea
- Picard, Michael H.
- Saffitz, Jeffrey E.
- Sanborn, Danita M. Yoerger
- Steinberg, Jonathan S.
- Tandri, Harikrishna
- Thiene, Gaetano
- Towbin, Jeffrey A.
- Tsatsopoulou, Adalena
- Venue
- Circulation
- Published
- 2010-04-06
- Volume
- 121
- Issue
- 13
- Pages
- 1533-1541
- ISSN
- 0009-7322 (ISSN-L)
- 1524-4539
- PMC
- PMC2860804
- OpenAlex
- W2131810639
- Cited by
- 2,676
- References
- 62
- Citations by year
- 2026: 64 · 2025: 83 · 2024: 96 · 2023: 86 · 2022: 108 · 2021: 232 · 2020: 230 · 2019: 176 · 2018: 193 · 2017: 207 · 2016: 204 · 2015: 219 · 2014: 202 · 2013: 182 · 2012: 179
- FWCI
- 124.40
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Digital Access to Scholarship at Harvard (DASH) (Harvard University) (submittedVersion, cc-by)
- UCL Discovery (University College London) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Funders
- European Commission
- National Institutes of Health
- National Heart, Lung, and Blood Institute (K23HL093350, R01HL086825, U01HL065594, U01HL065652, U01HL065691, R13 HL086825, R13HL086285)
- National Institute of Biomedical Imaging and Bioengineering (ZIAEB000072)
- NIH Clinical Center (ZIACL090019)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- arrhythmogenic right ventricular cardiomyopathy, diagnostic criteria, phenocopy, sudden cardiac death, familial disease, genetic diagnosis, electrocardiographic abnormalities, histopathology, cardiac arrhythmias, sensitivity and specificity
- MeSH terms
- Biopsy, Echocardiography, Humans, Magnetic Resonance Imaging, Reference Standards, Sensitivity and Specificity, Electrocardiography, Ambulatory, Death, Sudden, Cardiac, Practice Guidelines as Topic, Arrhythmogenic Right Ventricular Dysplasia
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