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Brugada Syndrome

 

Epidemiology

Clinical Features

ECG Findings

Syndrome Definition

Treatment

References

About This Document

 

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. ECG may show Brugada pattern.

         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.

         May be a history of chest pain, palpitation, syncope or family history of sudden death.

 

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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, T wave inversion, and no reciprocal findings.

         Pattern can come and go. Unmasked by fever, alcohol, cocaine, and some medications

 

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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.

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

 

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References:

         Audio Digest Emergency Medicine 2007. Vol 24(1)

         UpToDate Ver. 17.3 2011. Brugada syndrome and sudden cardiac death.

         Canadian Journal of Diagnosis. Feb. 2012