Brugada Syndrome

• Seen most commonly in young asian males
• "coved" ST elevations and RBBB in V1-V3
  • due to diminished inward sodium current in the region of the RV outflow tract epicardium
  • loss of the action potential dome in the RV epicardium due to unopposed ITo potassium outward current results in dramatic action potential shortening
  • The large potential difference between the normal endocardium and rapidly depolarized RV outflow epicardium gives rise to ST-segment elevation in V1-V3 in sinus rhythm
    • This predisposes to local reentry and ventricular arrhythmias
• Diagnosis is based on history and ECG but EP testing may be necessary for confirmation
  • Sodium channel-blocking drugs, such as procainamide and flecainide, can exacerbate the syndrome and are used as a provocative test
  • Other exacerbating factors: glucose followed by insulin, hypo or hypercalemia, febrile states, hi vagal states (sleep or rest)

Management of Confirmed Brugada Syndrome
ICD is the only effective treatment
ablation and pacers are being evaluated
antiarrhythmics have not shown benefit
Read the article attached for a nice review of the diagnosis and management from "brugada consensus" group

Long term prognosis: event rates in asymptomatic patients were low. Inducibility of ventricular tachyarrhythmia and family history of SCD are NOT predictors of cardiac events.

Outcome after ICD in patients with Brugada:In the above study, patients with type 1 Brugada ECG pattern were given ICD. a low incidence of arrhythmic events was found with an annual event rate of 1.6% over >3yrs, with 8.9% device-related complications/yr and frequent inappropriate shocks.

(Ellen Eaton MD, 8/13/10)