Authors
Antoine Andorin, Elijah R Behr, Isabelle Denjoy, Lia Crotti, Federica Dagradi, Laurence Jesel, Fréderic Sacher, Bertrand Petit, Philippe Mabo, Alice Maltret, Leonie CH Wong, Bruno Degand, Géraldine Bertaux, Philippe Maury, Yves Dulac, Béatrice Delasalle, Jean-Baptiste Gourraud, Dominique Babuty, Nico A Blom, Peter J Schwartz, Arthur A Wilde, Vincent Probst
Publication date
2016/6/1
Journal
Heart Rhythm
Volume
13
Issue
6
Pages
1274-1282
Publisher
Elsevier
Description
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 …
Total citations
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