We report on a youngster followed by his paediatrician from birth until 14 years of age for premature beats,most likely of ventricular origin. The sudden death of his sister provoked are-assessment of his electrocardiograms (ECG),resulting in the diagnosis of Brugada syndrome and the subsequent implantation of a cardioverter defibrillator. This syndrome is a well known entity in adult cardiology,first described by Brugada and Brugada in 1992. It is considered to be the second most common cause of death in young adults after road traffic accidents.In children,however,the Brugada syndrome is not well known and we believe to be certainly underdiagnosed.
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