Michowitz, Y;
Milman, A;
Andorin, A;
Sarquella-Brugada, G;
Gonzalez Corcia, MC;
Gourraud, J-B;
Conte, G;
Sacher, F;
Juang, JJM;
Kim, S-H;
et al.
Michowitz, Y; Milman, A; Andorin, A; Sarquella-Brugada, G; Gonzalez Corcia, MC; Gourraud, J-B; Conte, G; Sacher, F; Juang, JJM; Kim, S-H; Leshem, E; Mabo, P; Postema, PG; Hochstadt, A; Wijeyeratne, YD; Denjoy, I; Giustetto, C; Mizusawa, Y; Huang, Z; Jespersen, CH; Maeda, S; Takahashi, Y; Kamakura, T; Aiba, T; Arbelo, E; Mazzanti, A; Allocca, G; Brugada, R; Casado-Arroyo, R; Champagne, J; Priori, SG; Veltmann, C; Delise, P; Corrado, D; Brugada, J; Kusano, KF; Hirao, K; Calo, L; Takagi, M; Tfelt-Hansen, J; Yan, G-X; Gaita, F; Leenhardt, A; Behr, ER; Wilde, AAM; Nam, G-B; Brugada, P; Probst, V; Belhassen, B
(2019)
Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome.
J Am Coll Cardiol, 73 (14).
pp. 1756-1765.
ISSN 1558-3597
https://doi.org/10.1016/j.jacc.2019.01.048
SGUL Authors: Behr, Elijah Raphael
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Abstract
BACKGROUND: Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited. OBJECTIVES: The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence. METHODS: A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31). RESULTS: Patients' median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents. CONCLUSIONS: Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered.
Item Type: | Article |
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Additional Information: | © 2019. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ |
Keywords: | Brugada syndrome, SCN5A mutation, ablation, adolescence, pediatric, quinidine, Brugada syndrome, SCN5A mutation, ablation, adolescence, pediatric, quinidine, 1102 Cardiovascular Medicine And Haematology, 1117 Public Health And Health Services, Cardiovascular System & Hematology |
SGUL Research Institute / Research Centre: | Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) |
Journal or Publication Title: | J Am Coll Cardiol |
ISSN: | 1558-3597 |
Language: | eng |
Publisher License: | Creative Commons: Attribution-Noncommercial-No Derivative Works 4.0 |
PubMed ID: | 30975291 |
Go to PubMed abstract | |
URI: | https://openaccess.sgul.ac.uk/id/eprint/110809 |
Publisher's version: | https://doi.org/10.1016/j.jacc.2019.01.048 |
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