Follow-up from childhood to adulthood of individuals with family history of Brugada syndrome and normal electrocardiograms.

Follow-up FromChildhood to Adulthood of IndividualsWith Family History of Brugada Syndrome andNormal Electrocardiograms Brugada syndrome is an inherited primary arrhythmia syndromecharacterizedbycoved-typeST-segmentelevation inthe rightprecordial leadswithout structuralheartdiseaseand isassociatedwith increased riskof suddendeath.1 The syndrome is geneticallyandclinicallyheterogeneousandcanpresentwithin the firstmonthsof life, althoughmore typically in the fourthor fifthdecade.Thediagnostic type1electrocardiogram(ECG)may manifest spontaneously, increasing with age.2 Sex hormones have been suggested as potential responsible factors.3 Ajmaline challenge is recommended to unmask the diagnostic ECG pattern in patients with suspected disease.4 Although screening of first-degree relatives is common, no evidence-based guidelines exist, particularly for children with normal ECGs. We investigated the clinical significance of repeat testingafterpuberty inasymptomatic childrenwitha family history of Brugada syndrome who had an initial negative ajmaline test.