Andorin, A;
Behr, ER;
Denjoy, I;
Crotti, L;
Dagradi, F;
Jesel, L;
Sacher, F;
Petit, B;
Mabo, P;
Maltret, A;
et al.
Andorin, A; Behr, ER; Denjoy, I; Crotti, L; Dagradi, F; Jesel, L; Sacher, F; Petit, B; Mabo, P; Maltret, A; Wong, LC; Degand, B; Bertaux, G; Maury, P; Dulac, Y; Delasalle, B; Gourraud, JB; Babuty, D; Blom, NA; Schwartz, PJ; Wilde, AA; Probst, V
(2016)
Impact of clinical and genetic findings on the management of young patients with Brugada syndrome.
Heart Rhythm, 13 (6).
pp. 1274-1282.
ISSN 1556-3871
https://doi.org/10.1016/j.hrthm.2016.02.013
SGUL Authors: Behr, Elijah Raphael
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Available under License Creative Commons Attribution Non-commercial No Derivatives. Download (1MB) | Preview |
Abstract
BACKGROUND: Brugada syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) that seldom manifests or is recognized in childhood. OBJECTIVES: The objectives of this study were to describe the clinical presentation of pediatric BrS to identify prognostic factors for risk stratification and to propose a data-based approach management. METHODS: We studied 106 patients younger than 19 years at diagnosis of BrS enrolled from 16 European hospitals. RESULTS: At diagnosis, BrS was spontaneous (n = 36, 34%) or drug-induced (n = 70, 66%). The mean age was 11.1 ± 5.7 years, and most patients were asymptomatic (family screening, (n = 67, 63%; incidental, n = 13, 12%), while 15 (14%) experienced syncope, 6(6%) aborted SCD or symptomatic ventricular tachycardia, and 5 (5%) other symptoms. During follow-up (median 54 months), 10 (9%) patients had life-threatening arrhythmias (LTA), including 3 (3%) deaths. Six (6%) experienced syncope and 4 (4%) supraventricular tachycardia. Fever triggered 27% of LTA events. An implantable cardioverter-defibrillator was implanted in 22 (21%), with major adverse events in 41%. Of the 11 (10%) patients treated with hydroquinidine, 8 remained asymptomatic. Genetic testing was performed in 75 (71%) patients, and SCN5A rare variants were identified in 58 (55%); 15 of 32 tested probands (47%) were genotype positive. Nine of 10 patients with LTA underwent genetic testing, and all were genotype positive, whereas the 17 SCN5A-negative patients remained asymptomatic. Spontaneous Brugada type 1 electrocardiographic (ECG) pattern (P = .005) and symptoms at diagnosis (P = .001) were predictors of LTA. Time to the first LTA event was shorter in patients with both symptoms at diagnosis and spontaneous Brugada type 1 ECG pattern (P = .006). CONCLUSION: Spontaneous Brugada type 1 ECG pattern and symptoms at diagnosis are predictors of LTA events in the young affected by BrS. The management of BrS should become age-specific, and prevention of SCD may involve genetic testing and aggressive use of antipyretics and quinidine, with risk-specific consideration for the implantable cardioverter-defibrillator.
Item Type: | Article | ||||
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Additional Information: | © 2016. 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: | Arrhythmia, Brugada syndrome, Genetics, Pediatrics, Quinidine, Cardiovascular System & Hematology, 1102 Cardiovascular Medicine And Haematology, 0903 Biomedical Engineering | ||||
SGUL Research Institute / Research Centre: | Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cardiac (INCCCA) |
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Journal or Publication Title: | Heart Rhythm | ||||
ISSN: | 1556-3871 | ||||
Language: | eng | ||||
Dates: |
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Publisher License: | Creative Commons: Attribution-Noncommercial-No Derivative Works 4.0 | ||||
PubMed ID: | 26921764 | ||||
Go to PubMed abstract | |||||
URI: | https://openaccess.sgul.ac.uk/id/eprint/107737 | ||||
Publisher's version: | https://doi.org/10.1016/j.hrthm.2016.02.013 |
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