Management of Brugada syndrome in children

Abstract

Despite low prevalence in most populations, the Brugada syndrome has attracted considerable attention in recent years because of its propensity to cause sudden cardiac death in otherwise healthy young individuals, and because of pathophysiologic underpinnings that link it to other ‘ion channelopathies’ such as the congenital long-QT syndromes. The natural history of Brugada syndrome poses unique challenges to the management of children and adolescents diagnosed with the disorder, particularly among the substantial majority of pediatric patients who are entirely asymptomatic at presentation. This review will explore the current state of understanding of Brugada syndrome as it relates specifically to children and adolescents, and will provide an approach to management based on limited available data and existing consensus recommendations, which refer primarily to adult patients.