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

Overview Importance of Brugada Syndrome Importance of Brugada Syndrome ECG Changes in Brugada Syndrome ECG Changes in Brugada Syndrome Epidemiology Epidemiology Pathogenesis Pathogenesis Diagnosis Diagnosis Treatment Treatment ECG Practice 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 can be identified by ECG and successfully treated by ICD

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

Normal vs RBBB vs Brugada Syndrome Normal Normal RBBB RBBB –QRS ≥120ms –Terminal R wave in V1 (RSR 1 ) –Slurred S wave in I and V6 Brugada Syndrome Brugada Syndrome

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

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

What Type is It? AB CAB CAB CAB C

3 Different Patterns Feature Type 1 Type 2 Type 3 J wave ≥2mm≥2mm≥2mm T wave Negative Positive or biphasic Positive ST-TCovedSaddlebackSaddleback ST Segment Terminal Portion Gradual descent Elevated ≥1mm Elevated <1mm

Transient Nature

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

Pathogenesis Genetics Genetics –Autosomal dominant inheritance with variable expression –Cardiac sodium channel gene –No structural abnormalities

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

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

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

Outcome for Brugada Patients

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

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

Case 1 Male 62 years old presents to Foothills Male 62 years old presents to FoothillsHx Presented to family MD, asymptomatic Presented to family MD, asymptomatic No syncope No syncope No family history of sudden cardiac death No family history of sudden cardiac deathPMHx Htn, Hyperlipidemia Htn, Hyperlipidemia

RBBB + ST Elevation V1-V3 Type 1 Brugada ECG Pattern

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

Case 2 29 year old male Hx Cocaine use Cocaine use No personal or family hx of syncope, sudden cardiac death 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 ECG when not using cocaine normalized over several days IV Procainamide failed to produce Brugada changes IV Procainamide failed to produce Brugada changesDiagnosis Cocaine-induced ECG changes 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 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 Hx of sudden cardiac death in uncle at age 45 No hx of syncope No hx of syncopePMHx Healthy HealthyMeds No medications No medications

Subtle downsloping of ST in V1 and V2

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

Summary Think of Brugada syndrome in a patient with palpitations or syncope! 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. 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. Brugada. Brugada Syndrome: The Official Website of the Ramon Brugada Senior Foundation. Laszlo et al. Brugada-type electrocardiographic pattern induced by cocaine. Mayo Clin Proc. 2000;75: Laszlo et al. Brugada-type electrocardiographic pattern induced by cocaine. Mayo Clin Proc. 2000;75: &UID &UID Watrich et al. Brugada syndrome in a young patient with palpitations. CJEM 2005; 7(5): 347. Watrich et al. Brugada syndrome in a young patient with palpitations. CJEM 2005; 7(5): ED8A7848CD24B9A9C6 ED8A7848CD24B9A9C6 Wylie et al. Brugada syndrome and sudden cardiac arrest. Up To Date. June Wylie et al. Brugada syndrome and sudden cardiac arrest. Up To Date. June 2008.

Questions? Thanks for listening!