{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T07:40:44Z","timestamp":1768549244038,"version":"3.49.0"},"reference-count":14,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2020,10,21]],"date-time":"2020-10-21T00:00:00Z","timestamp":1603238400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,12,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The spectrum of inherited arrhythmogenic diseases (IADs) includes disorders without overt structural abnormalities (i.e. primary inherited arrhythmia syndromes) and structural heart diseases (i.e. arrhythmogenic ventricular cardiomyopathy, hypertrophic cardiomyopathy). The aim of this European Heart Rhythm Association (EHRA) survey was to evaluate current clinical practice and adherence to 2015 European Society of Cardiology Guidelines regarding the management of patients with IADs. A 24-item centre-based online questionnaire was presented to the EHRA Research Network Centres and the European Cardiac Arrhythmia Genetics Focus Group members. There were 46 responses from 20 different countries. The survey revealed that 37% of centres did not have any dedicated unit focusing on patients with IADs. Provocative drug challenges were widely used to rule-out Brugada syndrome (BrS) (91% of centres), while they were used in a minority of centres during the diagnostic assessment of long-QT syndrome (11%), early repolarization syndrome (12%), or catecholaminergic polymorphic ventricular tachycardia (18%). While all centres advised family clinical screening with electrocardiograms for all first-degree family members of patients with IADs, genetic testing was advised in family members of probands with positive genetic testing by 33% of centres. Sudden cardiac death risk stratification was straightforward and in line with current guidelines for hypertrophic cardiomyopathy, while it was controversial for other diseases (i.e. BrS). Finally, indications for ventricular mapping and ablation procedures in BrS were variable and not in agreement with current guidelines in up to 54% of centres.<\/jats:p>","DOI":"10.1093\/europace\/euaa223","type":"journal-article","created":{"date-parts":[[2020,7,10]],"date-time":"2020-07-10T19:10:57Z","timestamp":1594408257000},"page":"1904-1910","source":"Crossref","is-referenced-by-count":22,"title":["Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey"],"prefix":"10.1093","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2248-3456","authenticated-orcid":false,"given":"Giulio","family":"Conte","sequence":"first","affiliation":[{"name":"Division of Cardiology, Cardiocentro Ticino, Lugano, Switzerland"},{"name":"Faculty of Biomedical Sciences, USI, Lugano, Switzerland"},{"name":"Centre for Computational Medicine in Cardiology, Faculty of Informatics, Universit\u00e0 della Svizzera Italiana, Lugano, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Scherr","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Medical University of Graz, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Radoslaw","family":"Lenarczyk","sequence":"additional","affiliation":[{"name":"First Department of Cardiology and Angiology, Silesian Centre for Heart Disease, Zabrze, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Estelle","family":"Gandjbachkh","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Sorbonne Universit\u00e9s, APHP, Cardiology Institute, ICAN, Piti\u00e9-Salp\u00eatri\u00e8re University Hospital, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"St\u00e9phane","family":"Boul\u00e9","sequence":"additional","affiliation":[{"name":"Department of Cardiology, H\u00f4pital priv\u00e9 Le Bois, Lille, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael D","family":"Spartalis","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Onassis Cardiac Surgery Center, Athens, Greece"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elijah R","family":"Behr","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Cardiology Clinical Academic Group, St. George\u2019s, University of London and St. George\u2019s University Hospitals NHS Foundation Trust, London, UK"},{"name":"ERN GUARDHEART"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur","family":"Wilde","sequence":"additional","affiliation":[{"name":"ERN GUARDHEART"},{"name":"Amsterdam UMC, University of Amsterdam, Heart Center; Department of Clinical and Experimental Cardiology, Amsterdam Cardiovascular Sciences, Meibergdreef 9, 1105 AZ, Amsterdam, The 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