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

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Gonzalez, Maria Cecilia [UCL] de Asmundis, Carlo Chierchia, Gian-Battista Brugada, Pedro

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.

Document type Article de périodique (Journal article) – Synthèse de littérature
Publication date 2016
Language Anglais
Journal information "Cardiology in the Young" - Vol. 26, no.06, p. 1044-1055 (2016)
Peer reviewed yes
Publisher Cambridge University Press ((United Kingdom) Cambridge)
issn 1047-9511
e-issn 1467-1107
Publication status Publié
Affiliations UCL - SSS/IREC/SLUC - Pôle St.-Luc
UCL - (SLuc) Service de cardiologie pédiatrique
MESH Subject Brugada Syndrome ; Child ; Death, Sudden, Cardiac ; Defibrillators, Implantable ; Disease Management ; Electrocardiography ; Humans
Keywords Brugada syndrome ; Sodium channel mutations ; Sudden death ; Sudden infant death syndrome ; Syncope
Links
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Bibliographic reference Gonzalez, Maria Cecilia ; de Asmundis, Carlo ; Chierchia, Gian-Battista ; Brugada, Pedro. Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management. In: Cardiology in the Young, Vol. 26, no.06, p. 1044-1055 (2016)
Permanent URL http://hdl.handle.net/2078.1/184211