All::Cardiovascular System::Diseases::Brugada syndrome

Intro

What is Brugada syndrome?

a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy. It increases the risk of abnormal heart rhythms and sudden cardiac death.

How common is Brugada syndrome?

estimated prevalence of 1:5,000-10,000

What groups is Brugada syndrome more common?

Asians

around 20-40% of cases of Brugada syndrome are caused by a mutation in the {{c1::SCN5A gene which encodes the myocardial sodium ion channel protein}}

What are the ECG changes in Brugada syndrome?

  • convex ST segment elevation > 2mm in > 1 of V1-V3 followed by a negative T wave
  • partial right bundle branch block

What medications can be used to help diagnose Brugada syndrome?

flecainide or ajmaline

the ECG changes may be more apparent following the administration

What is the management of Brugada syndrome?

implantable cardioverter-defibrillator