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Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management

Published online by Cambridge University Press:  06 May 2016

M. Cecilia Gonzalez Corcia ,
Carlo de Asmundis ,
Gian-Battista Chierchia  and
Pedro Brugada
M. Cecilia Gonzalez Corcia*
Affiliation:
Pediatric Cardiology Department, Cliniques Universitaires St Luc, Brussels, Belgium
Carlo de Asmundis
Affiliation:
Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Belgium
Gian-Battista Chierchia
Affiliation:
Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Belgium
Pedro Brugada
Affiliation:
Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Belgium
*
Correspondence to: M. C. Gonzalez Corcia, Pediatric Cardiology Department, Cliniques Universitaires St Luc, Avenue de Hippocrates 10, 1200, Brussels, Belgium. Tel: +00 322 764 1380; Fax: +02 764 8911; E-mail: [email protected]
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Abstract

Brugada syndrome is an inherited arrhythmogenic disorder, characterised by coved-type ST-segment elevation in the right precordial leads, and is associated with increased risk of sudden death. It is genetically and clinically heterogeneous, presenting typically in the fourth or fifth decade of life. The prevalence of Brugada syndrome in the paediatric population is low compared with the adult population. Interestingly, over the last several years, there has been growing evidence in the literature of onset of the disease during childhood. Most of the paediatric cases reported in the literature consist of asymptomatic Brugada syndrome; however, some patients manifest the disease at different regions of the cardiac conduction system at a young age. Early expression of the disease can be affected by multiple factors, including genetic substrate, hormonal changes, and still unknown environmental exposures. The initial manifestation of Brugada syndrome in children can include sinus node dysfunction and atrial arrhythmias. Brugada syndrome can also manifest as ventricular arrhythmias leading to sudden death at an early age. In symptomatic children, performance of the ajmaline test by an experienced team can be safely used as a diagnostic tool to unmask latent Brugada syndrome. Defining indications for an implantable cardioverter defibrillator in children with the diagnosis of Brugada syndrome remains challenging. Given the rarity of the syndrome in children, most paediatric cardiologists will only rarely see a young patient with Brugada syndrome and there is still no universal consensus regarding the optimal management approach. Care should be individualised according to the specific clinical presentation, taking into account the family history, genetic data, and the family’s specific preferences.

Keywords

Brugada syndromesodium channel mutationssudden deathsudden infant death syndromesyncope
Type
Review Article
Information
Cardiology in the Young , Volume 26 , Issue 6 , August 2016 , pp. 1044 - 1055
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Copyright
© Cambridge University Press 2016 

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