Brugada syndrome

Question 1

Cause?

Answer 1

This is an inherited autosomal dominant cardiovascular disease

Question 2

Most common in which ethnicity?

Answer 2

Asians

Question 3

Pathophysiology?

Answer 3

Around 20-40% of cases are caused by a mutation in the SCN5A gene which encodes the myocardial sodium ion channel protein

Question 4

ECG changes?

Answer 4

• convex ST segment elevation > 2mm in > 1 of V1-V3 followed by an inverted T wave (known as Brugada’s sign)
• partial right bundle branch block
• the ECG changes may be more apparent following the administration of flecainide or ajmaline - this is the investigation of choice in suspected cases of Brugada syndrome

Question 5

Management?

Answer 5

Implantable cardioverter-defibrillator