Brugada Syndrome

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Title:              

Brugada Syndrome

Definition:

         Syncopal episodes and sudden death

ª       Associated with ECG changes:

Right bundle branch block

ST segment elevation in leads V1 – V3

Aetiology:

¨        Autosomal dominant inheritance

Pathogenesis

§        Abnormality in the cardiac sodium channel

         Episodic polymorphic ventricular tachycardia or ventricular fibrillation

Clinical Features:

History:

ª       Rare in the west, more common in South East Asia

¨        More common in young men

CVS:

§        Syncope

         Sudden death

Examination:

ª       No abnormalities on examination

Investigations:

Electrophysiology:

ECG

¨        Right bundle branch block

§         J point elevation and concave ST elevation in leads V1-V3

         If the ECG is normal the changes may be provoked by administration of ajmaline, procainamide, flecainide

Management:

Control

Surgery:

ª       Implantable cardioverter defibrillator

Prognosis:

¨        Poor without treatment

§        With treatment mortality reduced to 0 %

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