A form of inherited cardiovascular disease - may present with sudden 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

Risk factors:

Certain risk factors can increase the risk of arrhythmia. Patients should be warned to avoid them or take prompt action like taking paracetamol if any fever.

• Fever
• Excess alcohol intake
• Dehydration
• Medication
  • anti dysrhythmics like flecainide
  • verapamil
  • antidepressants like amitriptyline
• Electrolyte abnormalities

PC

• Patients may be asymptomatic or present with palpitations and syncope due to arrhythmias such as AV nodal re-entrant tachycardias (AVNRTs), VT or VF.

Investigations

ECG changes