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Brugada Syndrome

Brugada Syndrome. Carly Thompson MD CCFP EM Resident July 31, 2008. Overview. Importance of Brugada Syndrome ECG Changes in Brugada Syndrome Epidemiology Pathogenesis Diagnosis Treatment ECG Practice. Sudden Cardiac Arrest . Cardiac Arrest in a Structurally Normal Heart

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Brugada Syndrome

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  1. Brugada Syndrome Carly Thompson MD CCFP EM Resident July 31, 2008

  2. Overview Importance of Brugada Syndrome ECG Changes in Brugada Syndrome Epidemiology Pathogenesis Diagnosis Treatment ECG Practice

  3. Sudden Cardiac Arrest Cardiac Arrest in a Structurally Normal Heart • Long QT Syndrome • Preexcitation Syndrome • Commotio cordis • Brugada Syndrome

  4. Importance of Brugada Syndrome Mortality rate up to 10% / year in untreated patients with typical ECG changes!

  5. Importance of Brugada Syndrome • Brugada syndrome can be identified by ECG and successfully treated by ICD

  6. Brugada Syndrome: ECG Changes • First described in 1992 by J & P Brugada What is It? • Pseudo-RBBB • ST Elevation V1-V3

  7. Normal vs RBBB vs Brugada Syndrome • Normal • RBBB • QRS ≥120ms • Terminal R wave in V1 (RSR1) • Slurred S wave in I and V6 • Brugada Syndrome

  8. Pseudo- RBBB (but no slurred S in V6) • ST Elevation V1-V3 • T wave inversion = Brugada Syndrome ECG

  9. ST Patterns in Brugada Syndrome Type 1 “Coved Type” • J wave ≥ 2mm convex • ST segment descends • Inverted T wave Type 2 “Saddle back” • J wave ≥ 2mm • ST segment ≥1mm • Upright or biphasic T Type 3 “Saddle back” • J wave ≥2mm • ST segment <1mm • Positive T wave

  10. What Type is It? A B C

  11. 3 Different Patterns

  12. Transient Nature

  13. Epidemiology • Prevalence: • Japan 1.0%, Type 1 is common up to 0.16% • Finland 0.6%, Type 1 is rare • USA 0.4% • Gender: • Male (Up to 9x more common!) • Children • consider fever, syncope • Age • average age of diagnosis is 41

  14. Pathogenesis • Genetics • Autosomal dominant inheritance with variable expression • Cardiac sodium channel gene • No structural abnormalities

  15. Arrhythmias in Brugada Syndrome • Ventricular Arrhythmias • Localized reentry -> PVCs -> VT or VF • Atrial Fibrillation • More common!

  16. Sodium Imbalances Drugs: Cocaine TCAs and Neuroleptics in overdose Sodium channel blockers: procainamide Electrolyte Imbalances: Sodium, Calcium Lithium Drugs B-blockers Local anesthetics Autonomic Tone Fever Night Valsalva Pacing Provoking Factors

  17. Risk Factors for Sudden Cardiac Death • Male • Family history • Abnormal ECG • Inducible VT or VF • Previous syncope • 19% arrhythmia in 33 months • Previous arrest • 62% arrhythmia in 33 months

  18. Outcome for Brugada Patients

  19. Diagnosis Type 1 ECG changes + • Documented VF, VT • Family hx of sudden cardiac death • Family members with ECG changes • Inducible VT • Unexplained syncope probable VT/VF • Nocturnal agonal respiration Type 2 and 3 • Type 1 ECG induced with sodium channel blocker • And criteria above

  20. Treatment Quinidine • Less inducible VT Amiodarone • For patients with frequent discharges Implantable Cardioverter-Defibrillator (ICD) • Only treatment with proven efficacy

  21. Case 1 • Male 62 years old presents to Foothills Hx • Presented to family MD, asymptomatic • No syncope • No family history of sudden cardiac death PMHx • Htn, Hyperlipidemia

  22. RBBB + ST Elevation V1-V3Type 1 Brugada ECG Pattern

  23. Case 1 • Referred to electrophysiology for further testing, and possible ICD implantation.

  24. Case 2 29 year old male Hx • Cocaine use • No personal or family hx of syncope, sudden cardiac death

  25. RBBB, ST elevation V1-V3, T wave inversionBrugada-Type 1 ECG Changes

  26. Case 2 • ECG when not using cocaine normalized over several days • IV Procainamide failed to produce Brugada changes Diagnosis • Cocaine-induced ECG changes

  27. Case 3 29 year old Female Hx • Presented to the ER after 3 episodes of palpitations over 3 days, and a feeling of impending doom • Hx of sudden cardiac death in uncle at age 45 • No hx of syncope PMHx • Healthy Meds • No medications

  28. Subtle downsloping of ST in V1 and V2

  29. Case 3 • Cardiology consult: Patient was admitted to hospital • Procainamide challenge -> VT • ICD placed • Patient discharged home in stable condition

  30. Summary • Think of Brugada syndrome in a patient with palpitations or syncope! • Pseudo-RBBB • ST Elevation V1-V3 • Family history of sudden cardiac death • Send patients with suspicious ECGs to cardiology / electrophysiology for drug challenge or electrophysiology testing.

  31. References • Brugada. Brugada Syndrome: The Official Website of the Ramon Brugada Senior Foundation. http://www.brugada.org/ • Laszlo et al. Brugada-type electrocardiographic pattern induced by cocaine. Mayo Clin Proc. 2000;75:845-849. http://www.mayoclinicproceedings.com/inside.asp?AID=1503&UID • Watrich et al. Brugada syndrome in a young patient with palpitations. CJEM 2005; 7(5): 347. http://www.caep.ca/template.asp?id=D12C3F88B51A46ED8A7848CD24B9A9C6 • Wylie et al. Brugada syndrome and sudden cardiac arrest. Up To Date. June 2008.

  32. Questions? Thanks for listening!

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