Brugada SyndromeBrugada SyndromeCarly ThompsonCarly Thompson

MD CCFPMD CCFPEM ResidentEM ResidentJuly 31, 2008July 31, 2008

OverviewOverview

Importance of Brugada SyndromeImportance of Brugada Syndrome ECG Changes in Brugada SyndromeECG Changes in Brugada Syndrome EpidemiologyEpidemiology PathogenesisPathogenesis DiagnosisDiagnosis TreatmentTreatment ECG PracticeECG Practice

Sudden Cardiac ArrestSudden Cardiac Arrest

Cardiac Arrest in a Structurally Normal Cardiac Arrest in a Structurally Normal HeartHeart

– Long QT SyndromeLong QT Syndrome– Preexcitation SyndromePreexcitation Syndrome– Commotio cordisCommotio cordis– Brugada SyndromeBrugada Syndrome

Importance of Brugada Importance of Brugada SyndromeSyndrome

Mortality rate up to 10% / year in Mortality rate up to 10% / year in untreated patients with typical ECG untreated patients with typical ECG changes!changes!

Importance of Brugada Importance of Brugada SyndromeSyndrome

Brugada syndrome can be identified Brugada syndrome can be identified by ECG and successfully treated by by ECG and successfully treated by ICDICD

Brugada Syndrome: ECG Brugada Syndrome: ECG ChangesChanges

First described in 1992 by J & P First described in 1992 by J & P BrugadaBrugada

What is It?What is It? Pseudo-RBBBPseudo-RBBB ST Elevation V1-V3ST Elevation V1-V3

Normal vs RBBB vs Brugada Normal vs RBBB vs Brugada SyndromeSyndrome

NormalNormal

RBBBRBBB– QRS QRS ≥≥120ms120ms– Terminal R wave in V1 (RSRTerminal R wave in V1 (RSR11))– Slurred S wave in I and V6Slurred S wave in I and V6

Brugada SyndromeBrugada Syndrome

Pseudo- Pseudo- RBBB RBBB

(but no (but no slurred S slurred S in V6)in V6)

ST ST Elevation Elevation V1-V3V1-V3

T wave T wave inversioninversion

= Brugada = Brugada SyndromSyndrome ECG e ECG

ST Patterns in Brugada ST Patterns in Brugada SyndromeSyndrome

Type 1 “Coved Type”Type 1 “Coved Type” J wave J wave ≥ 2mm convex≥ 2mm convex ST segment descendsST segment descends Inverted T waveInverted T wave

Type 2 “Saddle back”Type 2 “Saddle back” J wave ≥ 2mmJ wave ≥ 2mm ST segment ≥1mmST segment ≥1mm Upright or biphasic TUpright or biphasic T

Type 3 “Saddle back”Type 3 “Saddle back” J wave ≥2mmJ wave ≥2mm ST segment <1mmST segment <1mm Positive T wavePositive T wave

What Type is It?What Type is It?

AA BB C C

3 Different Patterns3 Different Patterns

FeatureFeature Type 1Type 1 Type 2Type 2 Type 3Type 3

J waveJ wave ≥≥2mm2mm ≥≥2mm2mm ≥≥2mm2mm

T waveT wave NegativeNegative Positive or Positive or biphasicbiphasic

PositivePositive

ST-T ST-T CovedCoved SaddlebacSaddlebackk

SaddlebacSaddlebackk

ST ST Segment Segment Terminal Terminal PortionPortion

Gradual Gradual descentdescent

Elevated Elevated ≥1mm≥1mm

Elevated Elevated <1mm<1mm

Transient NatureTransient Nature

EpidemiologyEpidemiology

Prevalence:Prevalence:– Japan 1.0%, Type 1 is common up to 0.16%Japan 1.0%, Type 1 is common up to 0.16%– Finland 0.6%, Type 1 is rareFinland 0.6%, Type 1 is rare– USA 0.4%USA 0.4%

Gender: Gender: – Male (Up to 9x more common!)Male (Up to 9x more common!)

Children Children – consider fever, syncopeconsider fever, syncope

Age Age – average age of diagnosis is 41average age of diagnosis is 41

PathogenesisPathogenesis GeneticsGenetics

– Autosomal dominant inheritance with Autosomal dominant inheritance with variable expressionvariable expression

– Cardiac sodium channel geneCardiac sodium channel gene– No structural abnormalitiesNo structural abnormalities

Arrhythmias in Brugada Arrhythmias in Brugada SyndromeSyndrome

Ventricular ArrhythmiasVentricular Arrhythmias– Localized reentry -> PVCs -> VT or VFLocalized reentry -> PVCs -> VT or VF

Atrial FibrillationAtrial Fibrillation– More common!More common!

Provoking FactorsProvoking Factors

Sodium ImbalancesSodium Imbalances Drugs: CocaineDrugs: Cocaine TCAs and Neuroleptics TCAs and Neuroleptics

in overdosein overdose Sodium channel Sodium channel

blockers: blockers: procainamideprocainamide

Electrolyte Imbalances: Electrolyte Imbalances: Sodium, Calcium Sodium, Calcium

LithiumLithium

DrugsDrugs B-blockersB-blockers Local anestheticsLocal anesthetics

Autonomic ToneAutonomic Tone FeverFever NightNight ValsalvaValsalva PacingPacing

Risk Factors for Sudden Risk Factors for Sudden Cardiac DeathCardiac Death

MaleMale Family historyFamily history Abnormal ECGAbnormal ECG Inducible VT or VFInducible VT or VF Previous syncope Previous syncope

– 19% arrhythmia in 33 months19% arrhythmia in 33 months Previous arrestPrevious arrest

– 62% arrhythmia in 33 months62% arrhythmia in 33 months

Outcome for Brugada Outcome for Brugada PatientsPatients

DiagnosisDiagnosis

Type 1 ECG changes +Type 1 ECG changes + Documented VF, VTDocumented VF, VT Family hx of sudden cardiac deathFamily hx of sudden cardiac death Family members with ECG changesFamily members with ECG changes Inducible VTInducible VT Unexplained syncope probable VT/VFUnexplained syncope probable VT/VF Nocturnal agonal respirationNocturnal agonal respiration

Type 2 and 3Type 2 and 3 Type 1 ECG induced with sodium channel blockerType 1 ECG induced with sodium channel blocker And criteria aboveAnd criteria above

TreatmentTreatment

QuinidineQuinidine Less inducible VTLess inducible VT

AmiodaroneAmiodarone For patients with frequent dischargesFor patients with frequent discharges

Implantable Cardioverter-Defibrillator Implantable Cardioverter-Defibrillator (ICD)(ICD)

Only treatment with proven efficacyOnly treatment with proven efficacy

Case 1Case 1

Male 62 years old presents to FoothillsMale 62 years old presents to Foothills

HxHx Presented to family MD, asymptomaticPresented to family MD, asymptomatic No syncopeNo syncope No family history of sudden cardiac deathNo family history of sudden cardiac death

PMHxPMHx Htn, HyperlipidemiaHtn, Hyperlipidemia

RBBB + ST Elevation V1-V3RBBB + ST Elevation V1-V3Type 1 Brugada ECG PatternType 1 Brugada ECG Pattern

Case 1Case 1

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

Case 2Case 2

29 year old male29 year old male

Hx Hx Cocaine useCocaine use No personal or family hx of syncope, No personal or family hx of syncope,

sudden cardiac deathsudden cardiac death

RBBB, ST elevation V1-V3, T wave RBBB, ST elevation V1-V3, T wave inversioninversion

Brugada-Type 1 ECG ChangesBrugada-Type 1 ECG Changes

Case 2Case 2

ECG when not using cocaine ECG when not using cocaine normalized over several daysnormalized over several days

IV Procainamide failed to produce IV Procainamide failed to produce Brugada changesBrugada changes

DiagnosisDiagnosis Cocaine-induced ECG changesCocaine-induced ECG changes

Case 3Case 3

29 year old Female29 year old Female

HxHx Presented to the ER after 3 episodes of palpitations Presented to the ER after 3 episodes of palpitations

over 3 days, and a feeling of impending doomover 3 days, and a feeling of impending doom Hx of sudden cardiac death in uncle at age 45Hx of sudden cardiac death in uncle at age 45 No hx of syncopeNo hx of syncope

PMHxPMHx HealthyHealthy

MedsMeds No medicationsNo medications

Subtle downsloping of ST in V1 and V2Subtle downsloping of ST in V1 and V2

Case 3Case 3

Cardiology consult: Cardiology consult:

Patient was admitted to hospitalPatient was admitted to hospital Procainamide challenge -> VTProcainamide challenge -> VT ICD placedICD placed Patient discharged home in stable Patient discharged home in stable

conditioncondition

SummarySummary

Think of Brugada syndrome in a Think of Brugada syndrome in a patient with palpitations or syncope!patient with palpitations or syncope!– Pseudo-RBBBPseudo-RBBB– ST Elevation V1-V3ST Elevation V1-V3– Family history of sudden cardiac deathFamily history of sudden cardiac death

Send patients with suspicious ECGs Send patients with suspicious ECGs to cardiology / electrophysiology for to cardiology / electrophysiology for drug challenge or electrophysiology drug challenge or electrophysiology testing.testing.

ReferencesReferences

Brugada. Brugada Syndrome: The Official Website Brugada. Brugada Syndrome: The Official Website of the Ramon Brugada Senior Foundation.of the Ramon Brugada Senior Foundation.http://www.brugada.org/http://www.brugada.org/

Laszlo et al. Brugada-type electrocardiographic Laszlo et al. Brugada-type electrocardiographic pattern induced by cocaine. Mayo Clin Proc. pattern induced by cocaine. Mayo Clin Proc. 2000;75:845-849.2000;75:845-849.http://www.mayoclinicproceedings.com/inside.asp?http://www.mayoclinicproceedings.com/inside.asp?AID=1503&UIDAID=1503&UID

Watrich et al. Brugada syndrome in a young Watrich et al. Brugada syndrome in a young patient with palpitations. CJEM 2005; 7(5): 347.patient with palpitations. CJEM 2005; 7(5): 347.http://www.caep.ca/template.asp?http://www.caep.ca/template.asp?id=D12C3F88B51A46ED8A7848CD24B9A9C6id=D12C3F88B51A46ED8A7848CD24B9A9C6

Wylie et al. Brugada syndrome and sudden cardiac Wylie et al. Brugada syndrome and sudden cardiac arrest. Up To Date. June 2008.arrest. Up To Date. June 2008.

Questions?Questions?

Thanks for Thanks for listening!listening!