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Clinical aspects and physiopathology of Brugada syndrome: review of current concepts.

Publication Type:

Journal Article

Source:

Can J Physiol Pharmacol, Volume 84, Issue 8-9, p.795-802 (2006)

Keywords:

Brugada Syndrome, Electrocardiography, Female, Humans, Male, Muscle Proteins, Mutation, NAV1.5 Voltage-Gated Sodium Channel, Risk Factors, Sodium Channels

Abstract:

<p>Brugada syndrome (BS) is an inherited cardiac disorder characterized by typical electrocardiographic patterns of ST segment elevation in the precordial leads, right bundle branch block, fast polymorphic ventricular tachycardia in patients without any structural heart disease, and a high risk of sudden cardiac death. The incidence of BS is high in male vs. female (i.e., 8-10/1: male/female). The disorder is caused by mutations in the SCN5A gene encoding Nav1.5, the cardiac sodium channel, which is the only gene in which mutations were found to cause the disease. Mutations in SCN5A associated with the BS phenotype usually result in a loss of channel function by a reduction in Na+ currents. We review the clinical aspects, risk stratification, and therapeutic management of this important syndrome.</p>

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