Arrhythmogenic right ventricular cardiomyopathy
Desmosomal fibrofatty cardiomyopathy of young adults. T-wave inversion in V1 to V3, LBBB-morphology VT, cardiac MRI and Task Force criteria, exercise restriction, ICDs and family screening.
Tier 2Condition
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At a glance
- Inherited cardiomyopathy in which RV myocardium is replaced by fibrofatty tissue, causing VT and sudden death in young adults, often during exercise.
- Prevalence about 1 in 2,000 to 5,000. Presents in the second to fourth decade; men are affected more often and more severely.
- Clues: T-wave inversion in V1 to V3, LBBB-morphology VT or frequent ectopy, RV dilatation on imaging, a family history of young sudden death.
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Sources
- Arbelo E, Protonotarios A, Gimeno JR et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J 2023;44:3503-3626. doi:10.1093/eurheartj/ehad194European Society of Cardiology, 2023
- Zeppenfeld K, Tfelt-Hansen J, de Riva M et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J 2022;43:3997-4126. doi:10.1093/eurheartj/ehac262European Society of Cardiology, 2022
- Marcus FI, McKenna WJ, Sherrill D et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Circulation 2010;121:1533-1541Circulation, 2010
- Corrado D, Anastasakis A, Basso C et al. Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report. Int J Cardiol 2024;395:131447International Journal of Cardiology, 2024
- Assessing fitness to drive for commercial and private vehicle driversAU/NZAustroads and National Transport Commission, 2022
- Last updated
- 14 November 2026
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