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Friday 11 November 2011

Brugada syndrome

- first described in 1992
- more common in young male adults in South East Asia
- autosomal dominant
- approximately 20% of cases, the underlying cause is genetic defect in SCN5A gene, which encodes sodium channel controlling the depolarisation phase of cardiac action potential
- manifests with syncope and cardiac arrest, typically occurs in 3rd or 4th decade of life, usually at rest or during sleep
- ECG: ST elevation in leads V1 to V3 and RBBB (present spontaneously or provoked by ajmaline or flecainide)
- treatment: ICD


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