Arrhythmogenic right ventricular cardiomyopathy - What Is New since 2023 European Society of Cardiology Guidelines? Current Knowledge, Literature Review
DOI:
https://doi.org/10.12775/QS.2025.38.58173Keywords
Arrhythmogenic right ventricular cardiomyopathy, arrhythmogenic cardiomyopathy, ventricular arrhythmias, Task Force criteria for ARVC diagnosisAbstract
Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically conditioned disease, characterized by gradual fibro-fatty replacement of myocardium and tendency to ventricular arrhythmias and heart failure. ARVC is included in a group of inherited similar clinical entities characterized by similar remodeling changes, named in general arrhythmogenic cardiomyopathies (ACM). The new guidelines of European Society of Cardiology (ESC) for the management of cardiomyopathies (2023) redefine arrhythmogenic left ventricular cardiomyopathy (ALVC), left dominant ARVC, or arrhythmogenic dilated cardiomyopathy (DCM) as non-dilated left ventricular cardiomyopathy (NDLVC). The diagnostic process is based on Padua Criteria (2020) according to ESC guidelines and includes: right ventricle dysfunction evidence, endomyocardial biopsy results, defects of repolarization and conduction, arrhythmias and family history. However, in 2024 European Task Force consensus with modified guidelines was proposed. The treatment focuses on arrhythmias and heart failure management, prevention of sudden cardia death and family screening. Multidisciplinary approach is required to the proper management of the patients.
Aim of the study: The aim of the study is to review the current knowledge of ARVC, especially, novelties since 2023 ESC guidelines for the management of cardiomyopathies.
Material and methods: This article presents the current knowledge of the diagnosis of ARVC, especially on the basis of the guidelines of European Society of Cardiology and more recent experts’ consensus. Literature analysis, which contain recent report in ARVC diagnosis were analyzed using the PubMed platform. The search included the keywords: “arrhythmogenic right ventricle cardiomyopathy”, ,‚arrhythmogenic cardiomyopathy”, “Task Force criteria for ARVC diagnosis”, “ventricular arrhythmias”.
References
1. McKenna WJ, Thiene G, Nava A, et al. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J. 1994;71(3):215-218. doi:10.1136/hrt.71.3.215
2. 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(13):1533-1541. doi:10.1161/CIRCULATIONAHA.108.840827
3. Corrado D, Perazzolo Marra M, Zorzi A, et al. Diagnosis of arrhythmogenic cardiomyopathy: The Padua criteria. Int J Cardiol. 2020;319:106-114. doi:10.1016/j.ijcard.2020.06.005
4. Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503-3626. doi:10.1093/eurheartj/ehad194
5. Corrado D, Anastasakis A, Basso C, et al. Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report. Int J Cardiol. 2024;395:131447. doi:10.1016/j.ijcard.2023.131447
6. Thiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med. 1988;318(3):129-133. doi:10.1056/NEJM198801213180301
7. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30(6):1512-1520. doi:10.1016/s0735-1097(97)00332-x
8. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65(2):384-398. doi:10.1161/01.cir.65.2.384
9. Corrado D, Link MS, Calkins H. Arrhythmogenic Right Ventricular Cardiomyopathy. N Engl J Med. 2017;376(1):61-72. doi:10.1056/NEJMra1509267
10. Peters S, Trümmel M, Meyners W. Prevalence of right ventricular dysplasia-cardiomyopathy in a non-referral hospital. Int J Cardiol. 2004;97(3):499-501. doi:10.1016/j.ijcard.2003.10.037
11. Calkins H, Corrado D, Marcus F. Risk Stratification in Arrhythmogenic Right Ventricular Cardiomyopathy. Circulation. 2017;136(21):2068-2082. doi:10.1161/CIRCULATIONAHA.117.030792
12. Groeneweg JA, Bhonsale A, James CA, et al. Clinical Presentation, Long-Term Follow-Up, and Outcomes of 1001 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Patients and Family Members. Circ Cardiovasc Genet. 2015;8(3):437-446. doi:10.1161/CIRCGENETICS.114.001003
13. Gandjbakhch E, Redheuil A, Pousset F, Charron P, Frank R. Clinical Diagnosis, Imaging, and Genetics of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: JACC State-of-the-Art Review. J Am Coll Cardiol. 2018;72(7):784-804. doi:10.1016/j.jacc.2018.05.065
14. Te Riele AS, James CA, Philips B, et al. Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced. J Cardiovasc Electrophysiol. 2013;24(12):1311-1320. doi:10.1111/jce.12222
15. Towbin JA, McKenna WJ, Abrams DJ, et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. 2019;16(11):e301-e372. doi:10.1016/j.hrthm.2019.05.007
16. Castelletti S, Crotti L, Dagradi F, et al. Partial Pericardial Agenesis Mimicking Arrhythmogenic Right Ventricular Cardiomyopathy. Clin J Sport Med. 2020;30(5):e159-e162. doi:10.1097/JSM.0000000000000733
17. Laredo M, Duthoit G, Gandjbakhch E, Redheuil A, Hébert JL. Total pericardium agenesis mistaken for arrhythmogenic right ventricular cardiomyopathy. Eur Heart J Cardiovasc Imaging. 2018;19(1):120. doi:10.1093/ehjci/jex251
18. Tadros HJ, Miyake CY, Kearney DL, Kim JJ, Denfield SW. The Many Faces of Arrhythmogenic Cardiomyopathy: An Overview. Appl Clin Genet. 2023;16:181-203. Published 2023 Nov 1. doi:10.2147/TACG.S383446
19. Rootwelt-Norberg C, Lie ØH, Dejgaard LA, et al. Life-threatening arrhythmic presentation in patients with arrhythmogenic cardiomyopathy before and after entering the genomic era; a two-decade experience from a large volume center. Int J Cardiol. 2019;279:79-83. doi:10.1016/j.ijcard.2018.12.066
20. Platonov PG, Calkins H, Hauer RN, et al. High interobserver variability in the assessment of epsilon waves: Implications for diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia. Heart Rhythm. 2016;13(1):208-216. doi:10.1016/j.hrthm.2015.08.031
21. De Lazzari M, Zorzi A, Cipriani A, et al. Relationship Between Electrocardiographic Findings and Cardiac Magnetic Resonance Phenotypes in Arrhythmogenic Cardiomyopathy. J Am Heart Assoc. 2018;7(22):e009855. doi:10.1161/JAHA.118.009855
22. Pearman CM, Lee D, Davies B, et al. Incremental value of the signal-averaged ECG for diagnosing arrhythmogenic cardiomyopathy. Heart Rhythm. 2023;20(2):224-230. doi:10.1016/j.hrthm.2022.10.005
23. Corrado D, van Tintelen PJ, McKenna WJ, et al. Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis. Eur Heart J. 2020;41(14):1414-1429. doi:10.1093/eurheartj/ehz669
24. Hunold P, Wieneke H, Bruder O, et al. Late enhancement: a new feature in MRI of arrhythmogenic right ventricular cardiomyopathy?. J Cardiovasc Magn Reson. 2005;7(4):649-655. doi:10.1081/jcmr-65608
25. Cipriani A, Mattesi G, Bariani R, et al. Cardiac magnetic resonance imaging of arrhythmogenic cardiomyopathy: evolving diagnostic perspectives. Eur Radiol. 2023;33(1):270-282. doi:10.1007/s00330-022-08958-2
26. Réant P, Hauer AD, Castelletti S, et al. Epicardial myocardial strain abnormalities may identify the earliest stages of arrhythmogenic cardiomyopathy. Int J Cardiovasc Imaging. 2016;32(4):593-601. doi:10.1007/s10554-015-0813-9
27. Kirkels FP, Lie ØH, Cramer MJ, et al. Right Ventricular Functional Abnormalities in Arrhythmogenic Cardiomyopathy: Association With Life-Threatening Ventricular Arrhythmias. JACC Cardiovasc Imaging. 2021;14(5):900-910. doi:10.1016/j.jcmg.2020.12.028
28. Bluemke DA. ARVC: Imaging diagnosis is still in the eye of the beholder. JACC Cardiovasc Imaging. 2011;4(3):288-291. doi:10.1016/j.jcmg.2011.01.007
29. Aquaro GD, Barison A, Todiere G, et al. Usefulness of Combined Functional Assessment by Cardiac Magnetic Resonance and Tissue Characterization Versus Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy. Am J Cardiol. 2016;118(11):1730-1736. doi:10.1016/j.amjcard.2016.08.056
30. Tandri H, Saranathan M, Rodriguez ER, et al. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging. J Am Coll Cardiol. 2005;45(1):98-103. doi:10.1016/j.jacc.2004.09.053
31. Prati G, Vitrella G, Allocca G, et al. Right Ventricular Strain and Dyssynchrony Assessment in Arrhythmogenic Right Ventricular Cardiomyopathy: Cardiac Magnetic Resonance Feature-Tracking Study. Circ Cardiovasc Imaging. 2015;8(11):e003647. doi:10.1161/CIRCIMAGING.115.003647
32. Kellman P, Hernando D, Shah S, et al. Multiecho dixon fat and water separation method for detecting fibrofatty infiltration in the myocardium. Magn Reson Med. 2009;61(1):215-221. doi:10.1002/mrm.21657
33. Basha TA, Akçakaya M, Liew C, et al. Clinical performance of high-resolution late gadolinium enhancement imaging with compressed sensing. J Magn Reson Imaging. 2017;46(6):1829-1838. doi:10.1002/jmri.25695
34. Haugaa KH, Basso C, Badano LP, et al. Comprehensive multi-modality imaging approach in arrhythmogenic cardiomyopathy-an expert consensus document of the European Association of Cardiovascular Imaging. Eur Heart J Cardiovasc Imaging. 2017;18(3):237-253. doi:10.1093/ehjci/jew229
35. Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. 1997;30(6):1512-1520. doi:10.1016/s0735-1097(97)00332-x
36. Asimaki A, Saffitz JE. The role of endomyocardial biopsy in ARVC: looking beyond histology in search of new diagnostic markers. J Cardiovasc Electrophysiol. 2011;22(1):111-117. doi:10.1111/j.1540-8167.2010.01960.x
37. James CA, Jongbloed JDH, Hershberger RE, et al. International Evidence Based Reappraisal of Genes Associated With Arrhythmogenic Right Ventricular Cardiomyopathy Using the Clinical Genome Resource Framework. Circ Genom Precis Med. 2021;14(3):e003273. doi:10.1161/CIRCGEN.120.003273
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