Chang Gung medical journal
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A diagnostic triad characterizes Brugada syndrome. It consists of a right bundle branch block, ST-segment elevation in leads V1-V3 and sudden cardiac death (SCD). Approximately 50% of patients with Brugada syndrome noted to have familial occurrence, this suggests a genetic component of the disease. ⋯ With ICD therapy, the mortality rate at a 10 year follow-up was 0%. Supporting data for long-term pharmacological therapy with quinidine, or isoproterenol for prevention of SCD, in these patients, is uncomplete. Future advances in understanding the molecular mechanisms of Brugada syndrome may provide answers to many of the controversial issues in the management of this disease.