Brugada Syndorme, Long QT

Question 1

What is Brugada syndrome?

Answer 1

A form of inherited cardiovascular disease that 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.

Question 2

In which population is Brugada syndrome more common?

Answer 2

Asians

This implies a potential genetic or environmental predisposition in this demographic.

Question 3

What percentage of Brugada syndrome cases are caused by a mutation in the SCN5A gene?

Answer 3

20-40%

The SCN5A gene encodes the myocardial sodium ion channel protein.

Question 4

What are the characteristic ECG changes in Brugada syndrome?

Answer 4

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

These changes may be more apparent after the administration of specific medications.

Question 5

What is the investigation of choice in suspected cases of Brugada syndrome?

Answer 5

Administration of flecainide or ajmaline

This can help reveal the characteristic ECG changes.

Question 6

What is the primary management strategy for Brugada syndrome?

Answer 6

Implantable cardioverter-defibrillator

This device is used to prevent sudden cardiac death.

Question 7

What is a life threatening feature of Brugada?

Answer 7

Fever