Brugada syndrome – minimizing overdiagnosis and over treatment in children

PURPOSE OF REVIEW: Is to summarise the new contributions toward the understanding of the broad spectrum of manifestations of Brugada syndrome (BrS) during the first years of life. The review encompasses the screening of the asymptomatic patient referred due to family history in one extreme of the spectrum, and also the rare child with early clinical expression of the disease on the opposite side. RECENT FINDINGS: Involve specific features of pediatric BrS including the risk related to a positive family history of sudden cardiac death, the risk of presenting with syncope and the multiple diagnostic challenges of the disease. We included some of the most controversial aspects of the diagnosis and risk stratification, encompassing noninvasive studies (Holter monitors, exercise test, implantable loop recorders, and provocative tests), as well as invasive stratification during the first years of life. Finally, the role and concerns of genetic testing in this age group are commented upon. SUMMARY: The main key to minimize overdiagnosis and overtreatment in the young population with a personal and/or family diagnosis of BrS is to perform a systematic but also individualized assessment. Appropriate diagnostic guidelines need to be created and age-specific risk stratification algorithms built for the young patient both with suspected and confirmed BrS.