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      Arrhythmic risk stratification in arrhythmogenic right ventricular cardiomyopathy

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          Abstract

          Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable cardiomyopathy characterized by a predominantly arrhythmic presentation. It represents the leading cause of sudden cardiac death (SCD) among athletes and poses a significant morbidity threat in the general population. As a causative treatment for ARVC is still not available, the placement of an implantable cardioverter defibrillator represents the current cornerstone for SCD prevention in this setting. Thanks to international ARVC-dedicated efforts, significant steps have been achieved in recent years towards an individualized, patient-centred risk stratification approach. A novel risk calculator algorithm estimating the 5-year risk of arrhythmias of patients with ARVC has been introduced in clinical practice and subsequently validated. The purpose of this article is to summarize the body of evidence that has allowed the development of this tool and to discuss the best way to implement its use in the care of an individual patient.

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          Most cited references103

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          2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

          Katja Zeppenfeld, Jacob Tfelt‐Hansen, Marta de Riva … (2022)
          Article 0 comments Cited 325 times – based on 0 reviews     Review now
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            2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society

            Sana Al-Khatib, William G. Stevenson, Michael J Ackerman … (2018)
            Article 0 comments Cited 199 times – based on 0 reviews     Review now
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              2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy

              Jeffrey A. Towbin, William J. McKenna, Dominic J. Abrams … (2019)
              Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
              Article 0 comments Cited 198 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Alessio Gasperetti:
                ORCID: https://orcid.org/0000-0003-3432-070X
                Cynthia A James:
                ORCID: https://orcid.org/0000-0001-8040-4600
                Richard T Carrick:
                ORCID: https://orcid.org/0000-0001-5589-1216
                Alexandros Protonotarios:
                ORCID: https://orcid.org/0000-0001-8595-7212
                Anneline S J M te Riele:
                ORCID: https://orcid.org/0000-0002-4694-4790
                Julia Cadrin-Tourigny:
                ORCID: https://orcid.org/0000-0002-7489-8744
                Paolo Compagnucci:
                ORCID: https://orcid.org/0000-0003-1924-6548
                Firat Duru:
                ORCID: https://orcid.org/0000-0002-4748-0158
                Peter van Tintelen:
                ORCID: https://orcid.org/0000-0003-3854-6749
                Perry M Elliot:
                ORCID: https://orcid.org/0000-0003-3383-3984
                Hugh Calkins:
                ORCID: https://orcid.org/0000-0002-9262-9433
                Journal
                Journal ID (nlm-ta): Europace
                Journal ID (iso-abbrev): Europace
                Journal ID (publisher-id): europace
                Title: Europace
                Publisher: Oxford University Press (US )
                ISSN (Print): 1099-5129
                ISSN (Electronic): 1532-2092
                Publication date Collection: November 2023
                Publication date (Electronic): 03 November 2023
                Publication date PMC-release: 03 November 2023
                Volume: 25
                Issue: 11
                Electronic Location Identifier: euad312
                Affiliations
                Division of Cardiology, Department of Medicine, Johns Hopkins University , Blalock 545, 600 N. Wolfe St., Baltimore, MD 21287, USA
                Department of Genetics, University Medical Center Utrecht, University of Utrecht, Heidelberglaan 100, Utrecht , The Netherlands
                Department of Medicine, Division of Cardiology, University Medical Center Utrecht, Utrecht University, Heidelberglaan 100, Utrecht , Utrecht, The Netherlands
                Division of Cardiology, Department of Medicine, Johns Hopkins University , Blalock 545, 600 N. Wolfe St., Baltimore, MD 21287, USA
                Division of Cardiology, Department of Medicine, Johns Hopkins University , Blalock 545, 600 N. Wolfe St., Baltimore, MD 21287, USA
                Department of Cardiology, UCL Institute of Cardiovascular Science , London, UK
                Department of Medicine, Division of Cardiology, University Medical Center Utrecht, Utrecht University, Heidelberglaan 100, Utrecht , Utrecht, The Netherlands
                Cardiovascular Genetics Center, Montreal Heart Institute, Université de Montréal , Montréal, QC, Canada
                Cardiology and Arrhythmology Clinic, Marche University Hospital , Ancona, Italy
                Department of Cardiology, Arrhythmia Unit, University Heart Center, University Hospital Zurich , Zurich, Switzerland
                Department of Genetics, University Medical Center Utrecht, University of Utrecht, Heidelberglaan 100, Utrecht , The Netherlands
                Department of Cardiology, UCL Institute of Cardiovascular Science , London, UK
                Division of Cardiology, Department of Medicine, Johns Hopkins University , Blalock 545, 600 N. Wolfe St., Baltimore, MD 21287, USA
                Author notes
                Corresponding authors. Tel: 667-2127958, E-mail address: hcalkins@ 123456jhmi.edu (H.C.); alessio.gasperetti93@ 123456gmail.com (A.G.)
                [†]

                Member of the European Reference Network for rare, low prevalence and complex diseases of the heart: ERN GUARD-Heart (ERN GUARDHEART; http://guardheart.ern-net.eu).

                Conflict of interest: none declared.

                Author information
                https://orcid.org/0000-0003-3432-070X
                https://orcid.org/0000-0001-8040-4600
                https://orcid.org/0000-0001-5589-1216
                https://orcid.org/0000-0001-8595-7212
                https://orcid.org/0000-0002-4694-4790
                https://orcid.org/0000-0002-7489-8744
                https://orcid.org/0000-0003-1924-6548
                https://orcid.org/0000-0002-4748-0158
                https://orcid.org/0000-0003-3854-6749
                https://orcid.org/0000-0003-3383-3984
                https://orcid.org/0000-0002-9262-9433
                Article
                Publisher ID: euad312
                DOI: 10.1093/europace/euad312
                PMC ID: 10674106
                PubMed ID: 37935403
                SO-VID: 3406ceb6-8546-4059-9883-5cf6905712bb
                Copyright © © The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 06 May 2023
                Date accepted : 19 October 2023
                Page count
                Pages: 12
                Categories
                Subject: State of the Art Review
                Subject: AcademicSubjects/MED00200
                Subject: Eurheartj/1
                Subject: Eurheartj/7

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: arrhythmogenic right ventricular cardiomyopathy,risk stratification,primary prevention,icd
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: arrhythmogenic right ventricular cardiomyopathy, risk stratification, primary prevention, icd

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