Brugada Syndrome

 

Epidemiology

Clinical Features

ECG Findings

Syndrome Definition

Treatment

References

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Epidemiology:

·         ECG changes found in .4% of asymptomatic people in the USA.  Much higher in Southeast Asian populations.

·         4 to 5% of sudden cardiac deaths, predominantly those occurring at night and in young people.  Males predominate.

·         50% have an identified genetic component – probably a sodium channel gene.

·         20% of cardiac arrests where the heart is structurally normal.

 

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Clinical Features:

·         Most people are asymptomatic

·         Commonest presentation is syncope or sudden death.  Chest pain is not common.

·         Atrial fibrillation is more likely to occur.  Think about this diagnosis.

·         Pre-terminal rhythms most commonly polymorphic or monomorphic ventricular tachycardia leading to ventricular fibrillation.

·         Common contributor to cocaine-induced arrhythmias.

·         Possibly triggered by any drug blocking sodium channels including tricyclic overdose, psychotropics, flecanide and procainamide.

 

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ECG Findings:  (see Figure)

·         Polymorphic or monomorphic ventricular tachycardia when symptomatic.

·         Asymptomatic patients – usually changes in V1, V2, and sometimes V3.  Pseudo RBBB pattern (not seen in limb leads) with convex or concave ST elevation and no reciprocal findings.

 

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Syndrome Definition:

ECG findings plus at least 1 of the following:

·         Documented ventricular fibrillation

·         Self-terminating polymorphic ventricular tachycardia (VT)

·         Family history of sudden cardiac death at <45 years

·         Type 1 ST segment elevation in family members

·         Electrophysiologic inducibility of VT

·         Unexplained syncope suggestive of a tachyarrhythmia

·         Nocturnal agonal respiration

 

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Treatment:

·         Symptomatic – cardioversion or defibrillation based on ECG.  If polymorphic ventricular tachycardia remember to use unsynchronized shocks at 200-360 joules (monophasic).

·         Asymptomatic – refer quickly for electrophysiologic testing.  Avoid drugs which block sodium channels.  Most patients require and implantable cardioverter-defibrillator.

 

References:

·         Audio – Digest Emergency Medicine 2007.  Vol 24(1)

·         UpToDate Ver. 14.2  2006.  Brugada syndrome and sudden cardiac death.

 

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