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    Brugada SyndromeBrugada SyndromeCarly ThompsonCarly Thompson

    MD CCFPMD CCFPEM ResidentEM Resident

    July 31, !!"July 31, !!"

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    #$er$ie%#$er$ie%

    &mportan'e o( Brugada Syndrome&mportan'e o( Brugada Syndrome EC) Changes in Brugada SyndromeEC) Changes in Brugada Syndrome EpidemiologyEpidemiology PathogenesisPathogenesis DiagnosisDiagnosisTreatmentTreatment

    EC) Pra'ti'eEC) Pra'ti'e

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    Sudden Cardia' *rrestSudden Cardia' *rrest

    Cardia' *rrest in a Stru'turally +ormalCardia' *rrest in a Stru'turally +ormal

    earteart

    - .ong /T Syndrome.ong /T Syndrome

    - Pree0'itation SyndromePree0'itation Syndrome

    - Commotio 'ordisCommotio 'ordis

    - Brugada SyndromeBrugada Syndrome

    http://www.imageenvision.com/stock_clipart/details/0025-0802-2610-2956/red_love_heart_cartoon_character_dressed_as_a_super_hero
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    &mportan'e o( Brugada&mportan'e o( Brugada

    SyndromeSyndrome

    Mortality rate up to 1! 2 year inMortality rate up to 1! 2 year in

    untreated patients %ith typi'al EC)untreated patients %ith typi'al EC)

    'hanges'hanges

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    &mportan'e o( Brugada&mportan'e o( Brugada

    SyndromeSyndrome

    Brugada syndrome 'an 4e identi5edBrugada syndrome 'an 4e identi5ed

    4y EC) and su''ess(ully treated 4y4y EC) and su''ess(ully treated 4y

    &CD&CD

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    Brugada Syndrome6 EC)Brugada Syndrome6 EC)

    ChangesChanges

    First des'ri4ed in 177 4y J 8 PFirst des'ri4ed in 177 4y J 8 P

    BrugadaBrugada

    9hat is &t:9hat is &t: Pseudo;RBBBPseudo;RBBB

    ST Ele$ation

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    +ormal $s RBBB $s Brugada+ormal $s RBBB $s Brugada

    SyndromeSyndrome

    +ormal+ormal

    RBBBRBBB

    - /RS/RS ==1!ms1!ms

    - Terminal R %a$e in RSRTerminal R %a$e in RSR11??

    - Slurred S %a$e in & and

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    Pseudo;Pseudo;

    RBBBRBBB

    >4ut no>4ut no

    slurred Sslurred Sin

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    ST Patterns in BrugadaST Patterns in Brugada

    SyndromeSyndrome

    Type 1 Co$ed TypeType 1 Co$ed Type J %a$eJ %a$e = mm 'on$e0= mm 'on$e0 ST segment des'endsST segment des'ends &n$erted T %a$e&n$erted T %a$e

    Type Saddle 4a'Type Saddle 4a' J %a$e = mmJ %a$e = mm ST segment =1mmST segment =1mm pright or 4iphasi' Tpright or 4iphasi' T

    Type 3 Saddle 4a'Type 3 Saddle 4a' J %a$e =mmJ %a$e =mm ST segment 1mmST segment 1mm Positi$e T %a$ePositi$e T %a$e

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    9hat Type is &t:9hat Type is &t:

    ** BB CC

    http://en.wikipedia.org/wiki/Image:Brugada.jpghttp://en.wikipedia.org/wiki/Image:Brugada.jpg
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    3 DiGerent Patterns3 DiGerent Patterns

    FeatureFeature Type 1Type 1 Type Type Type 3Type 3

    J %a$eJ %a$e ==mmmm ==mmmm ==mmmm

    T %a$eT %a$e +egati$e+egati$e Positi$e orPositi$e or4iphasi'4iphasi' Positi$ePositi$e

    ST;TST;T Co$edCo$ed Saddle4a'Saddle4a'

    Saddle4a'Saddle4a'

    STSTSegmentSegment

    TerminalTerminalPortionPortion

    )radual)radualdes'entdes'ent

    Ele$atedEle$ated=1mm=1mm

    Ele$atedEle$ated1mm1mm

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    Transient +atureTransient +ature

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    EpidemiologyEpidemiology

    Pre$alen'e6Pre$alen'e6- Japan 1H!, Type 1 is 'ommon up to !H1@Japan 1H!, Type 1 is 'ommon up to !H1@

    - Finland !H@, Type 1 is rareFinland !H@, Type 1 is rare

    - S* !HIS* !HI )ender6)ender6

    - Male >p to 70 more 'ommon?Male >p to 70 more 'ommon?

    ChildrenChildren- 'onsider (e$er, syn'ope'onsider (e$er, syn'ope

    *ge*ge- a$erage age o( diagnosis is I1a$erage age o( diagnosis is I1

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    PathogenesisPathogenesis

    )eneti's)eneti's- *utosomal dominant inheritan'e %ith*utosomal dominant inheritan'e %ith

    $aria4le e0pression$aria4le e0pression

    - Cardia' sodium 'hannel geneCardia' sodium 'hannel gene- +o stru'tural a4normalities+o stru'tural a4normalities

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    *rrhythmias in Brugada*rrhythmias in Brugada

    SyndromeSyndrome

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    Pro$oing Fa'torsPro$oing Fa'tors

    Sodium ImbalancesSodium Imbalances

    Drugs6 Co'aineDrugs6 Co'aine

    TC*s and +eurolepti'sTC*s and +eurolepti's

    in o$erdosein o$erdose Sodium 'hannelSodium 'hannel

    4lo'ers6 pro'ainamide4lo'ers6 pro'ainamide

    Ele'trolyte &m4alan'es6Ele'trolyte &m4alan'es6

    Sodium, Cal'iumSodium, Cal'ium .ithium.ithium

    DrugsDrugs

    B;4lo'ersB;4lo'ers

    .o'al anestheti's.o'al anestheti's

    Autonomic ToneAutonomic Tone

    Fe$erFe$er

    +ight+ight

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    Ris Fa'tors (or SuddenRis Fa'tors (or Sudden

    Cardia' DeathCardia' Death

    MaleMale Family historyFamily history

    *4normal EC)*4normal EC) &ndu'i4le

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    #ut'ome (or Brugada#ut'ome (or Brugada

    PatientsPatients

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    DiagnosisDiagnosis

    Type 1 ECG changes +Type 1 ECG changes + Do'umented

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    TreatmentTreatment

    /uinidine/uinidine

    .ess indu'i4le

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    Case 1Case 1

    Male @ years old presents to FoothillsMale @ years old presents to Foothills

    00 Presented to (amily MD, asymptomati'Presented to (amily MD, asymptomati' +o syn'ope+o syn'ope +o (amily history o( sudden 'ardia' death+o (amily history o( sudden 'ardia' death

    PM0PM0 tn, yperlipidemiatn, yperlipidemia

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    RBBB N ST Ele$ation

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    Case 1Case 1

    Re(erred to ele'trophysiology (orRe(erred to ele'trophysiology (or

    (urther testing, and possi4le &CD(urther testing, and possi4le &CD

    implantationHimplantationH

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    Case Case

    7 year old male7 year old male

    00 Co'aine useCo'aine use

    +o personal or (amily h0 o( syn'ope,+o personal or (amily h0 o( syn'ope,

    sudden 'ardia' deathsudden 'ardia' death

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    RBBB, ST ele$ation

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    Case Case

    EC) %hen not using 'o'aineEC) %hen not using 'o'aine

    normalied o$er se$eral daysnormalied o$er se$eral days

    &< Pro'ainamide (ailed to produ'e&< Pro'ainamide (ailed to produ'eBrugada 'hangesBrugada 'hanges

    DiagnosisDiagnosis Co'aine;indu'ed EC) 'hangesCo'aine;indu'ed EC) 'hanges

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    Case 3Case 3

    7 year old Female7 year old Female

    00 Presented to the ER a(ter 3 episodes o( palpitationsPresented to the ER a(ter 3 episodes o( palpitations

    o$er 3 days, and a (eeling o( impending doomo$er 3 days, and a (eeling o( impending doom 0 o( sudden 'ardia' death in un'le at age IO0 o( sudden 'ardia' death in un'le at age IO +o h0 o( syn'ope+o h0 o( syn'ope

    PM0PM0 ealthyealthy

    MedsMeds +o medi'ations+o medi'ations

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    Su4tle do%nsloping o( ST in

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    Case 3Case 3

    Cardiology 'onsult6Cardiology 'onsult6

    Patient %as admitted to hospitalPatient %as admitted to hospital

    Pro'ainamide 'hallenge ;K

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    SummarySummary

    Thin o( Brugada syndrome in aThin o( Brugada syndrome in a

    patient %ith palpitations or syn'opepatient %ith palpitations or syn'ope

    - Pseudo;RBBBPseudo;RBBB

    - ST Ele$ation

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    Re(eren'esRe(eren'es

    BrugadaH Brugada Syndrome6 The #'ial 9e4site o(BrugadaH Brugada Syndrome6 The #'ial 9e4site o(the Ramon Brugada Senior FoundationHthe Ramon Brugada Senior FoundationH

    http622%%%H4rugadaHorg2http622%%%H4rugadaHorg2 .aslo et alH Brugada;type ele'tro'ardiographi'.aslo et alH Brugada;type ele'tro'ardiographi'

    pattern indu'ed 4y 'o'aineH Mayo Clin Pro'Hpattern indu'ed 4y 'o'aineH Mayo Clin Pro'H!!!QO6"IO;"I7H!!!QO6"IO;"I7H

    http622%%%Hmayo'lini'pro'eedingsH'om2insideHasp:http622%%%Hmayo'lini'pro'eedingsH'om2insideHasp:*&DA1O!38&D*&DA1O!38&D

    9atri'h et alH Brugada syndrome in a young patient9atri'h et alH Brugada syndrome in a young patient%ith palpitationsH CJEM !!O Q>O?6 3IQH%ith palpitationsH CJEM !!O Q>O?6 3IQH

    http622%%%H'aepH'a2templateHasp:http622%%%H'aepH'a2templateHasp:idAD1C3F""BO1*I@ED"*Q"I"CDIB7*7C@idAD1C3F""BO1*I@ED"*Q"I"CDIB7*7C@

    9ylie et alH Brugada syndrome and sudden 'ardia'9ylie et alH Brugada syndrome and sudden 'ardia'arrestH p To DateH June !!"HarrestH p To DateH June !!"H

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    /uestions:/uestions:

    Thans !orThans !orlistening"listening"


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