Cardiology Pearl: Brugada Syndrome

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Author: Puja Gopal, MSIV
Medical Student
University of Illinois School of Medicine

A 41-year-old otherwise healthy male presents to the emergency department after a syncopal episode while standing and waiting for the bus. He states he has been experiencing intermittent episodes of lightheadedness while at rest over the past several weeks. The patient has no other associated symptoms and no significant family history. He has no other complaints and a normal physical exam. His EKG is shown below.

EKG:

Brugada syndrome is a critical diagnosis for an emergency physician because left undiagnosed it may lead to cardiac arrest and death. Brugada syndrome is a disorder related to sodium channel dysfunction in the myocardium. It may be hereditary or due to a spontaneous mutation and occurs in otherwise structurally normal hearts. It most commonly occurs in males, especially those of Asian descent, and tends to present around 40 years of age. Estimated incidence is 0.05–0.60% in adults. Brugada is very rarely diagnosed in children.

It may be asymptomatic, cause nocturnal difficulty breathing, or present the first time as a syncopal episode or sudden cardiac death (SCD). Patients are prone to developing syncope or SCD due to ventricular tachyarrhythmias, primarily ventricular tachycardia or ventricular fibrillation.

The most specific EKG finding for Brugada is a prominent coved or down sloping ST segment elevation of >2mm in more than 1 lead in leads V1-V3 (often V1-V2), T wave inversion, and a RBBB or incomplete RBBB pattern. This is also known as Type 1. The other two types of Brugada are not diagnostic but can warrant further investigation in the right clinical context.

Currently, the only definitive treatment that has been proven effective in treating the ventricular arrhythmias and preventing SCD is an implantable cardioverter defibrillator (ICD). Thus, patients suspected of having Brugada need immediate cardiology consultation.

The patient presented above had the classic presentation and EKG findings and had an ICD placed the same day.

For more information on the diagnosis of Brugada Sydrome click here to view Dr. Amal Mattu’s Emergency ECG Video of the Week from 2012.

References:

1) Wilde A, Antzelevitch C, Borggrefe M, et al. Proposed diagnostic criteria for the Brugada syndrome: Consensus report. Circulation. 2002;106:2514-2519.

2) Mattau, Amal, Brugada syndrome. Amal Mattu’s Emergency ECG video of the week, http://ekgumem.tumblr.com/ March 4, 2012. Available at http://ekgumem.tumblr.com/post/18789986620/brugada-syndrome-episode. Accessed September 29, 2014.

3) Sheikh A, Ranjan K. Brugada syndrome: A review of the literature. Clinical Medicine. 2014; 14(5):482-489. doi: 10.7861/clinmedicine.14-5-482