Impact of clinical and genetic findings on the management of young patients with Brugada syndrome

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TitreImpact of clinical and genetic findings on the management of young patients with Brugada syndrome
Type de publicationJournal Article
Year of Publication2016
AuteursAndorin A, Behr ER, Denjoy I, Crotti L, Dagradi F, Jesel L, Sacher F, Petit B, Mabo P, Maltret A, Wong LCH, Degand B, Bertaux G, Maury P, Dulac Y, Delasalle B, Gourraud J-B, Babuty D, Blom NA, Schwartz PJ, Wilde AA, Probst V
JournalHEART RHYTHM
Volume13
Pagination1274-1282
Date PublishedJUN
Type of ArticleArticle
ISSN1547-5271
Mots-clésarrhythmia, Brugada syndrome, genetics, Pediatrics, Quinidine
Résumé

{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

DOI10.1016/j.hrthm.2016.02.013