Friday, 8 March 2019

Brugada syndrome


Brugada syndrome is a form of inherited cardiovascular disease with may present with sudden cardiac death. It is inherited in an autosomal dominant fashion and has an estimated prevalence of 1:5,000-10,000. Brugada syndrome is more common in Asians.

 

Pathophysiology

  • a large number of variants exist
  • around 20-40% of cases are caused by a mutation in the SCN5A gene which encodes the myocardial sodium ion channel protein

 

ECG changes

  • convex ST segment elevation > 2mm in > 1 of V1-V3 followed by a negative T wave
  • partial right bundle branch block
  • changes may be more apparent following flecainide
ECG showing Brugada pattern, most marked in V1, which has an incomplete RBBB, a downsloping ST segment and an inverted T wave

 

Management

  • implantable cardioverter-defibrillator

 

Complications

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