Conte, G; Scherr, D; Lenarczyk, R; Gandjbachkh, E; Boulé, S; Spartalis, MD; Behr, ER; Wilde, A; Potpara, T
(2020)
Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey.
Europace, 22 (12).
pp. 1904-1910.
ISSN 1532-2092
https://doi.org/10.1093/europace/euaa223
SGUL Authors: Behr, Elijah Raphael
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Abstract
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.
Item Type: |
Article
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Additional Information: |
This is a pre-copyedited, author-produced version of an article accepted for publication in EP Europace following peer review. The version of record Giulio Conte, Daniel Scherr, Radoslaw Lenarczyk, Estelle Gandjbachkh, Stéphane Boulé, Michael D Spartalis, Elijah R Behr, Arthur Wilde, Tatjana Potpara, Diagnosis, family screening, and treatment of inherited arrhythmogenic diseases in Europe: results of the European Heart Rhythm Association Survey, EP Europace, Volume 22, Issue 12, December 2020, Pages 1904–1910 is available online at: https://doi.org/10.1093/europace/euaa223 |
Keywords: |
Cardiomyopathies, EHRA survey, Genetic heart disease, Inherited arrhythmogenic diseases, Inherited primary arrhythmia syndromes, Sudden cardiac arrest, Sudden cardiac death, Sudden cardiac death, Sudden cardiac arrest, Inherited arrhythmogenic diseases, Inherited primary arrhythmia syndromes, Cardiomyopathies, Genetic heart disease, EHRA survey, 1103 Clinical Sciences, Cardiovascular System & Hematology |
SGUL Research Institute / Research Centre: |
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) |
Journal or Publication Title: |
Europace |
ISSN: |
1532-2092 |
Language: |
eng |
Dates: |
Date | Event |
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23 December 2020 | Published | 21 October 2020 | Published Online | 10 July 2020 | Accepted |
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Publisher License: |
Publisher's own licence |
PubMed ID: |
33367591 |
Web of Science ID: |
WOS:000606542700024 |
![](http://static.pubmed.gov/portal/portal3rc.fcgi/2745237/img/27842) |
Go to PubMed abstract |
URI: |
https://openaccess.sgul.ac.uk/id/eprint/112766 |
Publisher's version: |
https://doi.org/10.1093/europace/euaa223 |
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