Brugada syndrome is a genetic abnormality of the cardiac sodium channels that can lead to ventricular tachycardia and sudden cardiac death. It is characterized by ST elevation in leads V1-V3 of the electrocardiogram. While some patients are asymptomatic, others experience syncope typically due to nonsustained ventricular tachycardia. The only proven treatment that reduces the risk of sudden cardiac death is an implantable cardioverter defibrillator.

1. BRUGADA SYNDROMEA Brief Overview

2. What is Brugada Syndrome? Genetic abnormality of the cardiac sodium channels, leading to VT and/or SCD

3. Hereditary-60% of Brugada patients have a family history of SCD

5. A Few FactsCharacteristic EKG

6. Arrhythmias frequently occur during sleep

7. More common in certain ethnic groupsHow Common is Brugada?Difficult to know, due to recent discovery

8. First described at NASPE meeting in 1991

9. Review of literature estimates that Brugada causes 4-12% of unexpected sudden deaths worldwide -Plus up to 50% of SCD in patients with normal hearts

10. Patient CharacteristicsRelatively young

11. Predominantly male

12. No structural heart diseaseCommon EKG FindingsST elevation in V1-V3

13. Pseudo RBBBTypical Brugada EKG

14. Signs & SymptomsAsymptomatic

15. Syncope- typically due to NSVT -Check for family history of SCD -Perform PESSCD in otherwise young, healthy patientSigns & SymptomsCharacteristic EKG-In EKGs that appear normal, AADs can be given to provoke the typical findings -for patients with syncope of unknown origin or VF with unknown cause -also for patients with family history and first degree heart block in absence of ST elevation

16. AAD in Pts with Normal EKG

17. TreatmentOnly treatment that reduces risk of SCD is an ICD

18. Drug therapy has not been effective (beta blockers and amiodarone)IndicationsICD Recommended -Cardiac Arrest (Class I) -Documented VT (Class IIa) -Spontaneous ST elevation on EKG, and patient has had syncope (Class IIa)

19. HRS Update May 2011Risk Stratification and Therapy Selection in Brugada Syndrome- the PRELUDE RegistryIs inducible VT an accurate predictor of cardiac events in Brugada patients?

20. Results: VT/VF inducibility is not predictive of cardiac events; however, investigators found new risk indicators for arrhythmic eventsReferenceswww.brugada.orgwww.hrsonline.org