Left Ventricular Abnormal Substrate in Brugada Syndrome.
Brugada syndrome
SCN5A mutation
ventricular fibrillation
Journal
JACC. Clinical electrophysiology
ISSN: 2405-5018
Titre abrégé: JACC Clin Electrophysiol
Pays: United States
ID NLM: 101656995
Informations de publication
Date de publication:
10 2023
10 2023
Historique:
received:
09
03
2023
revised:
26
05
2023
accepted:
31
05
2023
medline:
27
10
2023
pubmed:
23
7
2023
entrez:
22
7
2023
Statut:
ppublish
Résumé
Slow-conductive structural abnormalities located in the epicardium of the right ventricle (RV) underlie Brugada syndrome (BrS). The extent of such substrate in the left ventricle (LV) has not been investigated. This study sought to characterize the extent of epicardial substrate abnormalities in BrS. We evaluated 22 consecutive patients (mean age 46 ± 11 years, 21 male) referred for recurrent ventricular arrhythmias (mean 10 ± 13 episodes) in the setting of BrS. The patients underwent clinical investigations and wide genetic screening to identify SCN5A mutations and common risk variants. High-density biventricular epicardial mapping was performed to detect prolonged (>70 ms) fragmented electrograms, indicating abnormal substrate area. All patients presented with abnormal substrate in the epicardial anterior RV (27 ± 11 cm A subset of patients with BrS present an abnormal substrate extending onto the LV epicardium and inferior RV that is associated with SCN5A mutations and multigenic variants.
Sections du résumé
BACKGROUND
Slow-conductive structural abnormalities located in the epicardium of the right ventricle (RV) underlie Brugada syndrome (BrS). The extent of such substrate in the left ventricle (LV) has not been investigated.
OBJECTIVES
This study sought to characterize the extent of epicardial substrate abnormalities in BrS.
METHODS
We evaluated 22 consecutive patients (mean age 46 ± 11 years, 21 male) referred for recurrent ventricular arrhythmias (mean 10 ± 13 episodes) in the setting of BrS. The patients underwent clinical investigations and wide genetic screening to identify SCN5A mutations and common risk variants. High-density biventricular epicardial mapping was performed to detect prolonged (>70 ms) fragmented electrograms, indicating abnormal substrate area.
RESULTS
All patients presented with abnormal substrate in the epicardial anterior RV (27 ± 11 cm
CONCLUSIONS
A subset of patients with BrS present an abnormal substrate extending onto the LV epicardium and inferior RV that is associated with SCN5A mutations and multigenic variants.
Identifiants
pubmed: 37480873
pii: S2405-500X(23)00353-5
doi: 10.1016/j.jacep.2023.05.039
pii:
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
2041-2051Commentaires et corrections
Type : CommentIn
Informations de copyright
Copyright © 2023 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Funding Support and Author Disclosures This work was supported by the National Research Agency (ANR-10-IAHU04-LIRYC), the European Research Council (FP7-SYMPHONY ERC-2012-ADG_20120314), the Leducq Foundation (Transatlantic Network of Excellence RHYTHM 16CVD02), and the Fondation Coeur et Artères (FCA17T2). Dr Gourraud has received a grant from the Fédération Française de Cardiologie, (PREVENT project). Dr Redon is supported by the National Agency for Research (ANR-GENSUD-14-CE10-0001). Dr Schott is supported by the Fondation Pour la Recherche Médicale (DEQ20140329545), IRP-VERACITIES—New Mechanisms for Ventricular Arrhythmia and Cardiometabolic Diseases, an I-SITE NExT Health and Engineering Initiative (Ecole Centrale and Nantes University), and IRP-GAINES—Genetic Architecture In Cardiovascular Diseases, funded by INSERM and CNRS. Dr Barc is supported by the research program Étoiles Montantes des Pays de la Loire (REGIOCARD RPH081-U1087-REG-PDL), ANR JCJC LEARN (R21006NN, RPV21014NNA), and the H2020-MSCA-IF-2014 Program of the European Commission (RISTRAD-661617). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.