AbstractThe Brugada syndrome (BS) is a relative new clinical entity characterized by complete or incomplete right bundle branch block (RBBB) and ST-segment elevation in leads V1 through V3 on surface electrocardiogram (ECG), the absence of structural heart disease, and a high risk of atrial fibrillation, ventricular tachycardia/ventricular fibrillation (VT/VF) and sudden cardiac death (SCD). The syndrome is considered responsible for 4-12% of all sudden deaths and at least 20% of deaths in patients with structurally normal hearts. BS typically manifests with syncope and cardiac arrest, occurring in the third and fourth decade of life, and usually at rest or during sleep. Although the average age at time of first diagnosis or SCD is 40 years, the BS has been described in a wide range of ages. The clinical phenotype is 8 to 10 times more prevalent in males than in females. Since its initial recognition, an exceptional progress regarding the pathophysiology and management of the disease has been performed... (excerpt)
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