Brugada Syndrome (BrS) is an inherited cardiac disorder, first identified by the Brugada brothers in 1992 to be a clinically distinct syndrome. Patients with BrS are at risk for sudden cardiac death, with generally no observed structural abnormalities observed in the heart. BrS is classically characterized by ST segment elevation in leads V1-V3 (for more review papers/background see some sample references below). Overall, the mechanism for disease progression is largely associated with mutations in the SCN5A sodium voltage gated channel.
This is a quick background on this rare disease. After reading these kinds of reviews for the disease as a whole, my focus shifted towards reports on paediatric BrS due to the research questions associated with the dataset I was working with. Stay tuned for more on how the story becomes quite interesting when we consider reports of BrS in children, and how the disease isn't identical as we know it in adults!
- Berne, P., & Brugada, J. (2012). Brugada syndrome 2012. Circulation Journal, 76(7), 1563-1571.
- Brugada, P., Brugada, R., & Brugada, J. (2000). The Brugada syndrome. Current Cardiology Reports, 2(6), 507-514.
- Brugada, P., & Brugada, J. (1992). Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report. Journal of the American College of Cardiology, 20(6), 1391-1396.