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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 – Long QT Syndrome – Preexcitation Syndrome – Commotio cordis – Brugada Syndrome Importance of Brugada Syndrome Mortality rate up to 10% / year in untreated patients with typical ECG changes! Importance of Brugada Syndrome Brugada syndrome can be identified by ECG and successfully treated by ICD Brugada Syndrome: ECG Changes First described in 1992 by J & P Brugada What is It? Pseudo-RBBB ST Elevation V1-V3 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 PseudoRBBB (but no slurred S in V6) ST Elevation V1-V3 T wave inversion = Brugada Syndrome ECG 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 What Type is It? A B C 3 Different Patterns Feature Type 1 Type 2 Type 3 J wave ≥2mm ≥2mm ≥2mm T wave Negative ST-T Coved Positive or Positive biphasic Saddleback Saddleback ST Segment Terminal Portion Gradual descent Elevated ≥1mm Elevated <1mm Transient Nature 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 Pathogenesis Genetics – Autosomal dominant inheritance with variable expression – Cardiac sodium channel gene – No structural abnormalities Arrhythmias in Brugada Syndrome Ventricular Arrhythmias – Localized reentry -> PVCs -> VT or VF Atrial Fibrillation – More common! Provoking Factors 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 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 Outcome for Brugada Patients 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 Treatment Quinidine Less inducible VT Amiodarone For patients with frequent discharges Implantable Cardioverter-Defibrillator (ICD) Only treatment with proven efficacy 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 RBBB + ST Elevation V1-V3 Type 1 Brugada ECG Pattern Case 1 Referred to electrophysiology for further testing, and possible ICD implantation. Case 2 29 year old male Hx Cocaine use No personal or family hx of syncope, sudden cardiac death RBBB, ST elevation V1-V3, T wave inversion Brugada-Type 1 ECG Changes Case 2 ECG when not using cocaine normalized over several days IV Procainamide failed to produce Brugada changes Diagnosis Cocaine-induced ECG changes 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 Subtle downsloping of ST in V1 and V2 Case 3 Cardiology consult: Patient was admitted to hospital Procainamide challenge -> VT ICD placed Patient discharged home in stable condition 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. 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=D12C3F88B51A46E D8A7848CD24B9A9C6 Wylie et al. Brugada syndrome and sudden cardiac arrest. Up To Date. June 2008. Questions? Thanks for listening!