Disease class: Cardiac conduction system disease
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
No comments:
Post a Comment