{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:31:08Z","timestamp":1790836268782,"version":"4.1.0"},"reference-count":51,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2024,12,2]],"date-time":"2024-12-02T00:00:00Z","timestamp":1733097600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,12,2]],"date-time":"2024-12-02T00:00:00Z","timestamp":1733097600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cardiovasc electrophysiol"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Arrhythmogenic cardiomyopathy (ACM) is a genetically heterogeneous inherited cardiomyopathy with an estimated prevalence of 1:5000\u201310\u2009000 that predisposes patients to life\u2010threatening ventricular arrhythmias (VA) and sudden cardiac death (SCD). ACM diagnostic criteria and risk prediction models, particularly for arrhythmogenic right ventricular cardiomyopathy (ARVC), the most common form of ACM, are typically genotype\u2010agnostic, but numerous studies have established clinically meaningful genotype\u2010phenotype associations. Early signs of ACM onset differ by genotype indicating the need for genotype\u2010specific diagnostic criteria and family screening paradigms. Likewise, risk factors for SCD vary by genetic subtype, indicating that genotype\u2010specific guidelines for management are also warranted. Of particular importance, genotype\u2010specific therapeutic approaches are being developed. Results from a randomized controlled trial for flecainide use in ARVC patients are currently pending. Research in a plakophilin\u20102\u2010deficient mouse model suggests this antiarrhythmic drug may be particularly useful for patients with likely pathogenic or pathogenic (LP\/P)\n                    <jats:italic>PKP2<\/jats:italic>\n                    variants. Additionally, the first gene therapy clinical trials in ARVC patients harboring LP\/P\n                    <jats:italic>PKP2<\/jats:italic>\n                    variants are currently underway. This review aims to provide clinicians caring for ACM patients with an up\u2010to\u2010date overview of the current literature in genotype\u2010specific natural history of disease and management of ACM patients and describe scientific advances that have led to upcoming clinical trials.\n                  <\/jats:p>","DOI":"10.1111\/jce.16519","type":"journal-article","created":{"date-parts":[[2024,12,3]],"date-time":"2024-12-03T00:28:38Z","timestamp":1733185718000},"page":"2662-2670","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Arrhythmogenic Cardiomyopathy: Towards Genotype Based Diagnoses and Management"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2674-8709","authenticated-orcid":false,"given":"Steven A.","family":"Muller","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine Johns Hopkins University Baltimore Maryland USA"},{"name":"Department of Cardiology University Medical Center Utrecht Utrecht The Netherlands"},{"name":"Member of the European Reference Network for Rare low prevalence and complex diseases of the heart: ERN GUARD\u2010Heart' Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgia","family":"Bertoli","sequence":"additional","affiliation":[{"name":"Leon H. Charney Division of Cardiology New York University Grossman School of Medicine New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Medical Biology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Centers University of Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3432-070X","authenticated-orcid":false,"given":"Alessio","family":"Gasperetti","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2825-8428","authenticated-orcid":false,"given":"Moniek G. P. J.","family":"Cox","sequence":"additional","affiliation":[{"name":"Department of Cardiology University Medical Centre Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9262-9433","authenticated-orcid":false,"given":"Hugh","family":"Calkins","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine Johns Hopkins University Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anneline S. J. 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