Brugada Syndrome: Rapid Review

Introduction

Brugada Syndrome was first described by Pedro and Josep Brugada in 1992 in eight otherwise healthy patients with sudden and aborted cardiac death, in whom they found “right bundle branch block and persistent ST segment elevation in leads V1 to V3”. It’s incidence seems to be particularly high in Southeast Asia where it had been previously described as Sudden Unexplained Nocturnal Death Syndrome (SUNDS), The condition is also believed to be one of the potential causes of sudden infant death syndrome and sudden cardiac death in young children. 

The condition is supposed to be due to a cardiac sodium channel mutation. ECG changes can be transient with Brugada syndrome and can also be unmasked or augmented by severe, schema and medications. 

Check out this short video on Brugada Syndrome, by the legendary "Amal Mattu" 

Different patterns of STE in Brugada Syndrome:

1) Coved: a coved-type (straight or convex upward) terminating in an inverted T-wave, more predictive of arrhythmic events.

2) Saddle: Concave upward

Diagnostic Criteria:

This ECG abnormality must be associated with one of the following clinical criteria to make the diagnosis:

• Documented ventricular fibrillation or polymorphic ventricular tachycardia
• Family history of sudden cardiac death at <45 years old 
• Coved-type ECGs in family members
• Inducibility of VT with programmed electrical stimulation 
• Syncope
• Nocturnal agonal respiration

Definite Treatment

Pharmacological treatment does not protect against recurrent events and implantation of an cardiac defibrillators is the only proven effective treatment in preventing sudden death in patients with the Brugada syndrome.

The term “Brugada Syndrome” is used when the Brugada ECG is accompanied by symptoms of syncope or cardiac arrest.

Take Home

• Knowledge of Brugada ECG pattern is paramount for Emergency Physicians.
• Patients with suspected Brugada Syndrome require an early cardiology or electrophysiology opinion. 
• Definite treatment is implantation of a defibrillator to prevent death from sudden ventricular arrhythmia.  

Further Reading:

1. http://lifeinthefastlane.com/what-is-brugada-syndrome/
2. https://www.youtube.com/watch?v=0pbGlhefScg (Part 2)
3. http://www.medscape.com/viewarticle/828939