Definition and treatment of arrhythmogenic cardiomyopathy: an updated expert panel report
Elliott, Perry M.; Anastasakis, Aris; Asimaki, Angeliki; Basso, Cristina; Bauce, Barbara; Brooke, Matthew A.; Calkins, Hugh; Corrado, Domenico; Duru, Firat; Green, Kathleen J.; Judge, Daniel P.; Kelsell, David; Lambiase, Pier D.; McKenna, William J.; Pilichou, Kalliopi; Protonotarios, Alexandros; Saffitz, Jeffrey E.; Syrris, Petros; Tandri, Hari; Te Riele, Anneline; Thiene, Gaetano; Tsatsopoulou, Adalena; van Tintelen, J. Peter
(2019) European Journal of Heart Failure, volume 21, issue 8, pp. 955 - 964
(Article)
Abstract
It is 35 years since the first description of arrhythmogenic right ventricular cardiomyopathy (ARVC) and more than 20 years since the first reports establishing desmosomal gene mutations as a major cause of the disease. Early advances in the understanding of the clinical, pathological and genetic architecture of ARVC resulted in
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consensus diagnostic criteria, which proved to be sensitive but not entirely specific for the disease. In more recent years, clinical and genetic data from families and the recognition of a much broader spectrum of structural disorders affecting both ventricles and associated with a propensity to ventricular arrhythmia have raised many questions about pathogenesis, disease terminology and clinical management. In this paper, we present the conclusions of an expert round table that aimed to summarise the current state of the art in arrhythmogenic cardiomyopathies and to define future research priorities.
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Keywords: Arrhythmogenic cardiomyopathy, Arrhythmogenic right ventricular cardiomyopathy, Ventricular arrhythmias, Cardiology and Cardiovascular Medicine, Journal Article
ISSN: 1388-9842
Publisher: Oxford University Press
Note: Funding Information: This project was an independent initiative supported by the Fondazione Internazionale Menarini, which had no part in designing or approving the agenda. St. Bartholomew's Hospital, the Amsterdam UMC and the Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua-Azienda Ospedaliera are members of ERN GUARD-HEART (European Reference Network for Rare and Complex Diseases of the Heart; http://guardheart.ern-net.eu), which endorsed the meeting. Conflict of interest: H.C. reports grants and personal fees from Boston Scientifiic, and personal fees from Medtronic, St Jude Medical, during the conduct of the study. D.P.J. reports grants from Menarini Foundation, during the conduct of the study; personal fees from Alnylam, GlaxoSmithKline, Pfizer, outside the submitted work. H.T. reports personal fees from Abbott, Cooltech, outside the submitted work. All other authors have nothing to disclose. Funding Information: This project was an independent initiative supported by the Fon-dazione Internazionale Menarini, which had no part in designing or approving the agenda. St. Bartholomew’s Hospital, the Amsterdam UMC and the Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua-Azienda Ospedaliera are members of ERN GUARD-HEART (European Reference Network for Rare and Complex Diseases of the Heart; http://guardheart.ern-net.eu), which endorsed the meeting. Conflict of interest: H.C. reports grants and personal fees from Boston Scientifiic, and personal fees from Medtronic, St Jude Medical, during the conduct of the study. D.P.J. reports grants from Menarini Foundation, during the conduct of the study; personal fees from Alnylam, GlaxoSmithKline, Pfizer, outside the submitted work. H.T. reports personal fees from Abbott, Cooltech, outside the submitted work. All other authors have nothing to disclose. Publisher Copyright: © 2019 The Authors. European Journal of Heart Failure © 2019 European Society of Cardiology
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