Brugada Syndrome

Brugada Syndrome is an inherited disorder associated with sudden cardiac death first described in the 1990s. The EKG is characterized by a right bundle branch block pattern and associated ST segment elevation in leads V1 and V2. The appearance of the ST abnormality is a sharply elevated ST segment followed by a down-sloping sine wave. It is believed to account for 4% of nonischemic sudden cardiac death and up to 20% of all sudden death in individuals with a structurally normal heart. Patients who inherit Brugada Syndrome seem to be predisposed to polymorphic ventricular tachycardia. The inherited gene(s) result in a mutation in the cardiac sodium channels.

An EKG demonstrating changes associated with Brugada Syndome

References:

Mattu, A et al. Electrocardiography in Emergency Medicine. 2007. ACEP Publishing