Brugada syndrome.

The syndrome of right bundle branch block, ST segment elevation from VI-V3 and syncopal episodes and/or sudden death in a patient with structurally normal heart is a new entity, named Brugada syndrome in 1992. In some individuals, the presentation may be asymptomatic with only abnormal electrocardographs while in others a history of sudden death in the family and electrocardiographic criteria are observed. This disease is genetically determined caused by a defect in the myocardial sodium channel (SCN5A). Sudden death in individuals with this syndrome can only be prevented by implanting a cardioverter-defibrillator.