Brugada Syndrome in Children

In recent years, numerous asymptomatic individuals worldwide have undergone electrophysiological studies “only” because they have a pathological ECG indicative of Brugada syndrome.1 Furthermore, at least 1 of 3 of these individuals had inducible ventricular fibrillation (VF) and underwent implantation of a cardioverter defibrillator (ICD).2 “Asymptomatic Brugada syndrome with inducible VF” became an accepted indication for ICD implantation because data from a single large study showed that 12% of such patients develop spontaneous VF within 3 years of diagnosis.3 More recent (albeit smaller) studies, however, suggested that the risk for spontaneous VF in asymptomatic patients is lower.4 In fact, the most recent studies show that only 3%5 to 4%6 of individuals undergoing ICD implantation for “asymptomatic Brugada syndrome with inducible VF” developed spontaneous VF within 3 years of implantation. At the same time, as many as 28% of them developed ICD-related complications.6 The realization that we have done more harm than good to many asymptomatic individuals has reopened the debate on the optimal management of asymptomatic Brugada syndrome. Indeed, defining the role of electrophysiological testing in asymptomatic Brugada syndrome is probably the most heated debate in arrhythmology nowadays.7,8 Fortunately, children have been spared from this debate because Brugada syndrome has been reported only rarely in minors, at least until now.Article p 2042 In this issue of Circulation , Probst et al9 describe 30 children and adolescents (≤16 years of age) with Brugada syndrome. Their mean age was 8±5 years; 22 of them were <12 years of age. The circumstances leading to diagnosis of Brugada syndrome are important; in only 11 children (37%) was the Brugada-type ECG recognized during an evaluation for arrhythmia-related symptoms (cardiac arrest in 1, syncope in 10).9 The majority of children (60%) were asymptomatic, and their Brugada-type …