{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T06:14:00Z","timestamp":1790316840693,"version":"4.1.0"},"reference-count":28,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2024,3,1]],"date-time":"2024-03-01T00:00:00Z","timestamp":1709251200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,3,1]],"date-time":"2024-03-01T00:00:00Z","timestamp":1709251200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,1,3]],"date-time":"2024-01-03T00:00:00Z","timestamp":1704240000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002996","name":"Netherlands Heart Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002996","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100024370","name":"Governo Italiano Ministero dell'Istruzione dell'Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100024370","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jacc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["JACC: Advances"],"published-print":{"date-parts":[[2024,3]]},"DOI":"10.1016\/j.jacadv.2024.100832","type":"journal-article","created":{"date-parts":[[2024,2,1]],"date-time":"2024-02-01T23:10:15Z","timestamp":1706829015000},"page":"100832","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":48,"title":["Long-Term Arrhythmic Follow-Up and\u00a0Risk\u00a0Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular\u00a0Cardiomyopathy"],"prefix":"10.1016","volume":"3","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3432-070X","authenticated-orcid":false,"given":"Alessio","family":"Gasperetti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5589-1216","authenticated-orcid":false,"given":"Richard","family":"Carrick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandros","family":"Protonotarios","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikael","family":"Laredo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"van der Schaaf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2363-8758","authenticated-orcid":false,"given":"Petros","family":"Syrris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brittney","family":"Murray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Crystal","family":"Tichnell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Cappelletto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Gigli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristen","family":"Medo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9523-6694","authenticated-orcid":false,"given":"Peter","family":"Crabtree","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ardan M.","family":"Saguner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Firat","family":"Duru","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robyn","family":"Hylind","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic","family":"Abrams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neal K.","family":"Lakdawala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Massie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7489-8744","authenticated-orcid":false,"given":"Julia","family":"Cadrin-Tourigny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattia","family":"Targetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iacopo","family":"Olivotto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maddalena","family":"Graziosi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moniek","family":"Cox","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Biagini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Charron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Casella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Tondo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Momina","family":"Yazdani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James S.","family":"Ware","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjay","family":"Prasad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonardo","family":"Cal\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Helms","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Hespe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4846-7676","authenticated-orcid":false,"given":"Jodie","family":"Ingles","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harikrishna","family":"Tandri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7891-3385","authenticated-orcid":false,"given":"Flavie","family":"Ader","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luisa","family":"Mestroni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arthur","family":"Wilde","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Merlo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Gandjbakhch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hugh","family":"Calkins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anneline S.J.M.","family":"te Riele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Peter van Tintelen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Perry","family":"Elliot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia A.","family":"James","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"7","key":"10.1016\/j.jacadv.2024.100832_bib1","doi-asserted-by":"crossref","first-page":"806","DOI":"10.1093\/eurheartj\/ehq025","article-title":"Diagnosis of arrhythmogenic right ventricular cardiomyopathy\/dysplasia: proposed modification of the task force criteria","volume":"31","author":"Marcus","year":"2010","journal-title":"Eur Heart J"},{"issue":"11","key":"10.1016\/j.jacadv.2024.100832_bib2","doi-asserted-by":"crossref","first-page":"e301","DOI":"10.1016\/j.hrthm.2019.05.007","article-title":"2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy","volume":"16","author":"Towbin","year":"2019","journal-title":"Heart Rhythm"},{"issue":"46","key":"10.1016\/j.jacadv.2024.100832_bib3","first-page":"3227","article-title":"Treatment of arrhythmogenic right ventricular cardiomyopathy\/dysplasia: an international task force consensus statement","volume":"36","author":"Corrado","year":"2015","journal-title":"Eur Heart J"},{"issue":"32","key":"10.1016\/j.jacadv.2024.100832_bib4","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1093\/eurheartj\/ehac180","article-title":"A\u00a0new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy","volume":"43","author":"Cadrin-Tourigny","year":"2022","journal-title":"Eur Heart J"},{"issue":"5","key":"10.1016\/j.jacadv.2024.100832_bib5","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1093\/europace\/euz352","article-title":"Long-term follow-up analysis of a highly characterized arrhythmogenic cardiomyopathy cohort with classical and non-classical phenotypes\u2013a real-world assessment of a novel prediction model: does the subtype really matter","volume":"22","author":"Casella","year":"2020","journal-title":"Europace"},{"issue":"8","key":"10.1016\/j.jacadv.2024.100832_bib6","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1016\/j.hrthm.2020.03.007","article-title":"Novel risk calculator performance in athletes with arrhythmogenic right ventricular cardiomyopathy","volume":"17","author":"Gasperetti","year":"2020","journal-title":"Heart Rhythm"},{"issue":"22","key":"10.1016\/j.jacadv.2024.100832_bib7","doi-asserted-by":"crossref","first-page":"2753","DOI":"10.1016\/j.jacc.2020.04.023","article-title":"Prognostic value of magnetic resonance phenotype in patients with arrhythmogenic right ventricular cardiomyopathy","volume":"75","author":"Aquaro","year":"2020","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"8","key":"10.1016\/j.jacadv.2024.100832_bib8","first-page":"1263","article-title":"External validation of a risk prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy","volume":"37","author":"Baudinaud","year":"2021","journal-title":"Can J Cardiol"},{"issue":"7","key":"10.1016\/j.jacadv.2024.100832_bib9","first-page":"1973","article-title":"Validation of an arrhythmogenic right ventricular cardiomyopathy risk-prediction model in a Chinese cohort","volume":"11","author":"Zhang","year":"2022","journal-title":"J\u00a0Clin Med"},{"issue":"32","key":"10.1016\/j.jacadv.2024.100832_bib10","doi-asserted-by":"crossref","first-page":"3053","DOI":"10.1093\/eurheartj\/ehac235","article-title":"Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator","volume":"43","author":"Protonotarios","year":"2022","journal-title":"Eur Heart J"},{"issue":"32","key":"10.1016\/j.jacadv.2024.100832_bib11","doi-asserted-by":"crossref","first-page":"3041","DOI":"10.1093\/eurheartj\/ehac289","article-title":"Arrhythmic risk prediction in arrhythmogenic right ventricular cardiomyopathy: external validation of the arrhythmogenic right ventricular cardiomyopathy risk calculator","volume":"43","author":"Jord\u00e0","year":"2022","journal-title":"Eur Heart J"},{"issue":"3","key":"10.1016\/j.jacadv.2024.100832_bib12","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCGEN.120.003273","article-title":"An international evidence based reappraisal of genes associated with arrhythmogenic right ventricular cardiomyopathy (ARVC) using the ClinGen framework","volume":"14","author":"James","year":"2021","journal-title":"Circ Genom Precis Med"},{"issue":"1","key":"10.1016\/j.jacadv.2024.100832_bib13","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1161\/CIRCULATIONAHA.120.053033","article-title":"Evidence-based assessment of genes in dilated cardiomyopathy","volume":"144","author":"Jordan","year":"2021","journal-title":"Circulation"},{"issue":"23","key":"10.1016\/j.jacadv.2024.100832_bib14","doi-asserted-by":"crossref","first-page":"1872","DOI":"10.1161\/CIRCULATIONAHA.119.044934","article-title":"Desmoplakin cardiomyopathy, a fibrotic and inflammatory form of cardiomyopathy distinct from typical dilated or arrhythmogenic right ventricular cardiomyopathy","volume":"141","author":"Smith","year":"2020","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.jacadv.2024.100832_bib15","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1093\/europace\/euab183","article-title":"Clinical characteristics and risk stratification of desmoplakin cardiomyopathy","volume":"24","author":"Wang","year":"2022","journal-title":"EP Europace"},{"issue":"14","key":"10.1016\/j.jacadv.2024.100832_bib16","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1093\/eurheartj\/ehu509","article-title":"Impact of genotype on clinical course in arrhythmogenic right ventricular dysplasia\/cardiomyopathy-associated mutation carriers","volume":"36","author":"Bhonsale","year":"2015","journal-title":"Eur Heart J"},{"issue":"5","key":"10.1016\/j.jacadv.2024.100832_bib17","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med"},{"issue":"1","key":"10.1016\/j.jacadv.2024.100832_bib18","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCEP.120.008509","article-title":"Sudden cardiac death prediction in arrhythmogenic right ventricular cardiomyopathy: a multinational collaboration","volume":"14","author":"Cadrin-Tourigny","year":"2021","journal-title":"Circ Arrhythm Electrophysiol"},{"issue":"8","key":"10.1016\/j.jacadv.2024.100832_bib19","doi-asserted-by":"crossref","first-page":"1315","DOI":"10.1016\/j.hrthm.2022.04.015","article-title":"Clinical profile and long-term follow-up of a cohort of patients with desmoplakin cardiomyopathy","volume":"19","author":"Bariani","year":"2022","journal-title":"Heart Rhythm"},{"issue":"25","key":"10.1016\/j.jacadv.2024.100832_bib20","doi-asserted-by":"crossref","first-page":"2175","DOI":"10.1016\/j.jacc.2008.09.019","article-title":"Left-dominant arrhythmogenic cardiomyopathy","volume":"52","author":"Sen-Chowdhry","year":"2008","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"7","key":"10.1016\/j.jacadv.2024.100832_bib21","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1016\/j.hrthm.2018.01.031","article-title":"Predicting arrhythmic risk in arrhythmogenic right ventricular cardiomyopathy: a systematic review and meta-analysis","volume":"15","author":"Bosman","year":"2018","journal-title":"Heart Rhythm"},{"issue":"21","key":"10.1016\/j.jacadv.2024.100832_bib22","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1161\/CIRCULATIONAHA.117.030792","article-title":"Risk stratification in arrhythmogenic right ventricular cardiomyopathy","volume":"136","author":"Calkins","year":"2017","journal-title":"Circulation"},{"issue":"6","key":"10.1016\/j.jacadv.2024.100832_bib23","doi-asserted-by":"crossref","first-page":"4080","DOI":"10.1002\/ehf2.13019","article-title":"Comparison of different prediction models for the indication of implanted cardioverter defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy","volume":"7","author":"Aquaro","year":"2020","journal-title":"ESC Heart Fail"},{"issue":"2","key":"10.1016\/j.jacadv.2024.100832_bib24","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1093\/europace\/euab162","article-title":"Comparing clinical performance of current implantable cardioverter-defibrillator implantation recommendations in arrhythmogenic right ventricular cardiomyopathy","volume":"24","author":"Bosman","year":"2022","journal-title":"EP Europace"},{"issue":"40","key":"10.1016\/j.jacadv.2024.100832_bib25","doi-asserted-by":"crossref","first-page":"3997","DOI":"10.1093\/eurheartj\/ehac262","article-title":"2022 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death","volume":"43","author":"Zeppenfeld","year":"2022","journal-title":"Eur Heart J"},{"issue":"21","key":"10.1016\/j.jacadv.2024.100832_bib26","doi-asserted-by":"crossref","first-page":"1981","DOI":"10.1016\/j.jacc.2022.08.804","article-title":"Prognostic prediction of genotype vs phenotype in genetic cardiomyopathies","volume":"80","author":"Paldino","year":"2022","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"21","key":"10.1016\/j.jacadv.2024.100832_bib27","doi-asserted-by":"crossref","first-page":"1995","DOI":"10.1016\/j.jacc.2022.09.018","article-title":"Genotype-based risk stratification can outperform phenotype-based practice for inherited cardiomyopathies","volume":"80","author":"James","year":"2022","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"29","key":"10.1016\/j.jacadv.2024.100832_bib28","doi-asserted-by":"crossref","first-page":"2842","DOI":"10.1093\/eurheartj\/ehab294","article-title":"Prediction of ventricular arrhythmia in phospholamban p.Arg14del mutation carriers\u2013reaching the frontiers of individual risk prediction","volume":"42","author":"Verstraelen","year":"2021","journal-title":"Eur Heart J"}],"container-title":["JACC: Advances"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2772963X24000085?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2772963X24000085?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,9]],"date-time":"2025-09-09T13:58:41Z","timestamp":1757426321000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2772963X24000085"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3]]},"references-count":28,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["S2772963X24000085"],"URL":"https:\/\/doi.org\/10.1016\/j.jacadv.2024.100832","relation":{},"ISSN":["2772-963X"],"issn-type":[{"value":"2772-963X","type":"print"}],"subject":[],"published":{"date-parts":[[2024,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Long-Term Arrhythmic Follow-Up and\u00a0Risk\u00a0Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular\u00a0Cardiomyopathy","name":"articletitle","label":"Article Title"},{"value":"JACC: Advances","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jacadv.2024.100832","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.","name":"copyright","label":"Copyright"}],"article-number":"100832"}}