Brugada Syndrome

Key points

• Inherited (autosomal dominant) cardiovascular disease that may present with sudden cardiac death; prevalence ~1:5,000–10,000; more common in Asians.
• Around 20–40% of cases are caused by a mutation in the SCN5A gene encoding the myocardial sodium ion channel protein.
• ECG: convex ST segment elevation >2 mm in >1 of V1–V3 followed by a negative T wave, with partial right bundle branch block; changes may be more apparent following flecainide.
• Management: implantable cardioverter-defibrillator (ICD).