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Educational Background: University of Santo Tomas, 1995 Santo Tomas Univ. Hospital, 1996  Post Graduate Training: Residency Training in Internal Medicine –UP PGH Fellowship in Adult Cardiology- UP PGH Training in Clinical Cardiac Electrophysiology and Pacing -Philippine Heart Center  Present Positions: Consultant, Philippine Heart Center  ERDIE C. FADREGUILAN, MD FPCP, FPCC ERDIE C. FADREGUILAN, MD FPCP, FPCC

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    Educational Background:University of Santo Tomas, 1995

    Santo Tomas Univ. Hospital, 1996

    Post Graduate Training:

    Residency Training in Internal Medicine UP PGHFellowship in Adult Cardiology- UP PGH

    Training in Clinical Cardiac Electrophysiology and Pacing

    -Philippine Heart Center

    Present Positions:Consultant, Philippine Heart Center

    ERDIE C. FADREGUILAN, MDFPCP, FPCC

    ERDIE C. FADREGUILAN, MDFPCP, FPCC

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    SUDDEN CARDIAC DEATH

    Erdie C. Fadreguilan, MD

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    SUDDEN CARDIAC DEATH

    Definition:

    Natural death due to cardiaccauses heralded by abrupt loss ofconsciousness within one hour ofthe onset of acute symptoms

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    SCA- A Public Health Issue

    AIDS2

    Breast

    Cancer1

    LungCancer1

    SCD3

    SCD claims more

    lives each year thanthese other diseasescombined:

    The Facts: Significant Killer in America

    95% fatal without ICDprotection5 - 98% survivalwith ICD protection

    Nearly 1000 SCA deaths/day6

    ICDs are Class I indicated formost at-risk Pts7

    New guidelines clearly outline

    at-risk groups7

    Studies show that ICDs arecost effective8

    14,000

    41,400

    335,000162,500

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    GROUP

    Patients with highcoronary-risk profile

    Patients with previouscoronary event

    Patients with ejectionfraction

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    What Is SCA?

    Electrical system in heart malfunctions

    Heart unexpectedly, abruptly stops beating

    Often caused by an abnormal heart rhythmcalled ventricular fibrillation (VF)

    VF accounts for half of all cardiac deaths

    Rapid, chaotic heartbeat

    Lower heart chambers, or ventricles,spasm

    Heart functioning stops

    Lack of oxygen in body, brain is dead

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    Etiology of Sudden Cardiac Death

    An estimated 13 million people had coronary heart disease (CHD) in the U.S. in 2002.1

    Sudden death was the first manifestation of CHD in 50% of men and 63% of women.1

    CHD accounts for at least 80% of sudden cardiac deaths in Western cultures.3

    1 American Heart Association. Heart Disease and Stroke Statistics2003 Update. Dallas, Tex.: American HeartAssociation; 2002.

    2 Adapted from Heikki et al. N Engl J Med, Vol. 345, No. 20, 2001.

    3 Myerberg RJ. Heart Disease, A Textbook of Cardiovascular Medicine. 6th ed. P. 895.

    Etiology of Sudden Cardiac Death2,3

    * ion-channel abnormalities, valvular or congenital heart disease, othercauses

    80%Coronary

    HeartDisease

    15%Cardiomyopathy

    5% Other*

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    SUDDEN CARDIAC DEATH

    Epidemiology SCD due to Coronary Artery

    disease: single most importantcause of death in adult population

    Ventricular Fibrillation: 75-80%Bradyarrhythmias: minority

    SCD in the absence of CAD, CHF:

    5-10% of cases

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    Arrhythmic Cause of SCD

    Albert CM. Circulation. 2003;107:2096-2101.

    12%Other CardiacCause

    88%

    ArrhythmicCause

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    PrimaryPrimary

    VFVF

    8%8%

    Underlying Arrhythmia of Sudden CardiacArrest

    Adapted from Bays de Luna A. Am Heart J. 1989;117:151-159.

    TorsadesTorsades

    de Pointesde Pointes

    13%13%

    Bradycardia17%

    VTVT

    62%62%

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    Sustained Monomorphic VT

    72-year-old woman with CHD

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    VF with Defibrillation (12-lead ECG)

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    Torsades de Pointes

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    SCA Survival & MortalityData

    At least 335,000 SCA deaths in the U.S.each year

    Only 5 to 10%survive first episode of SCA

    Roughly two-thirds of SCA deaths occurout-of-hospital 5% estimated survival rate

    Seidl K, Senges J. Card Electrophysiol Rev. 2003;7:5-13.

    Heart Disease and Stroke Statistics 2005 Update. AHA. www.americanheart.org

    Crespo EM, Kim J, Selzman KA. Am J Med Sci. 2005;329:238-246.

    Zheng ZJ, et al. Circulation. 2001;104:2158-2163.

    Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484.

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    Cummins RO. Annals Emerg Med. 1989;18:1269-1275.

    SCA Resuscitation Success vs.Time*

    10

    20

    30

    40

    50

    60

    70

    80

    90

    100

    0 1 2 3 4 5 6 7 8 9

    %

    Success

    *Non-linear

    Time (minutes)

    Chance of success reduced7 - 10% each minute

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    Can SCA be Prevented?

    Research and clinical studies haveidentified several conditions that putpatients at a high risk for SCA

    Patients can be evaluated for these known

    risk factors before they experience a SCA

    Treatment options are available that canprevent sudden cardiac death

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    Risk Factors for Sudden CardiacArrest

    Previous Sudden Cardiac Arrest (SCA) Event Prior Episode of Ventricular Tachyarrhythmia

    (VT)

    Previous Myocardial Infarction (MI)

    Coronary Artery Disease (CAD) Heart Failure

    Genetic diseases

    Long QT Syndrome

    Hypertrophic Cardiomyopathy (HCM) Brugada Syndrome

    ARVD

    SUDS

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    Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine, 5th ed, Vol 1.Philadelphia: WB Saunders Co;1997:ch 24.Fogoros RN. Practical Cardiac Diagnosis: Electrophysiologic Testing, 2nd ed. Blackwell Science, pp 172.The AVID Investigators. N Engl J Med. 1997;337:1576-1583.Myerburg RJ. Ann Intern Med.. 1993;119:1187-1197.Demirovic J. Progr Cardiovasc Dis. 1994;37:39-48.Friedlander Y. Circulation. 1998;97:155-160.

    Sudden Cardiac Arrest Survivors

    Highest risk factor for Sudden Cardiac Arrest is

    a previous SCA event

    30 to 50% of SCA survivors will experienceanother SCA event within one year

    First-degree relatives of SCA patients have a50% higher risk of MI or primary cardiac arrest

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    Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine, 5th ed, Vol 1.Philadelphia: WB Saunders Co;1997:ch 24.Fogoros RN. Practical Cardiac Diagnosis: Electrophysiologic Testing, 2nd ed. Blackwell Science, pp172.The AVID Investigators. N Engl J Med. 1997;337:1576-1583.

    Prior Episode of VT

    VT with syncope or a low ejection

    fraction (LVEF < 40%) leads to anincreased risk of Sudden Cardiac Arrest

    The one-year risk of Sudden CardiacArrest for these patients ranges from 20to 50%

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    SCA Relationship to MI

    A previous MI can be identified in asmany as 75% of SCA patients.

    A previous MI as a single risk-factorraises the one-year risk of SCA by 5%.

    The five-year risk of SCA is 32% forpatients with all of these risk-factors: history of MI

    non-sustained, inducible, non-suppressibleVT

    LVEF 40%

    Sudden Cardiac Arrest Fast Facts. HRS. www.hrsonline.org

    Risk factors for sudden cardiac death. www.heartinstitute.org.au/Community/scdMain.asp

    Buxton AE, et al. N Engl J Med. 1999;341:1882-1890.

    Ti D d f M t lit Ri k P t MI

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    Time Dependence of Mortality Risk Post-MIPrediction of Sudden Cardiac Death After Myocardial Infarction

    in the Beta-Blocking Era1

    700 post-MI patients;~ 95% on beta-blockers2 years after discharge.

    The epidemiologicpattern of SCD wasdifferent from thatreported in previousstudies.

    Arrhythmia events didnot concentrate earlyafter the index event;

    most occurred > 18months post-MI.

    1 Huikuri HV. J Am Coll Cardiol. 2003;42:652-658.

    Total

    Mortality

    CardiacMortality

    Non-SCD

    SCDCumulativ e

    Events(%)

    18

    15

    12

    9

    6

    3

    18

    15

    12

    9

    6

    3

    20

    40

    60 20

    40

    60

    Follow-Up (months) Follow-Up (months)

    l i f i f C fi

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    14

    11.6

    8.47.89

    8.2

    4.9

    7.2

    0

    2

    4

    6

    8

    10

    12

    14

    16

    1-17 mo 18 - 59 mo 60 - 119 mo > 120 mo

    Conv

    ICD

    (n =300)

    (n =283)

    (n =284)

    (n =292)Hazard

    Ratio.98

    (p = 0.92)

    0.52

    (p = 0.07)

    0.50

    (p = 0.02)

    0.62

    (p = 0.09)

    Wilber, D. Circulation. 2004;109:1082-1084.

    Relation of Time from MI to ICD Benefitin the MADIT-II Trial

    Time from MI%

    Mortalityf or

    Each

    T

    imeP

    eriod

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    SCD Rates in Post-MI Patientswith LV Dysfunction

    2119.8

    14

    10

    7

    16 16

    12 9.4

    28

    1820

    28

    0

    10

    20

    30

    TRACE CAPRICORN EMIAT MADIT MUSTT

    Inducible

    MUSTT

    Registry

    MADIT II*

    ControlGroupMortalityat2year

    Total Mortality

    Arrhythmic Mortality

    Total Mortality ~20-30%; SCDaccounts for ~50% of the total deaths.

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    People whove had a heart attackhave a sudden death rate that is 4-6

    times that of the general population.1

    People who have had a heart attackand have LV dysfunction (

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    SCD in Heart Failure 1, 2

    Despite improvements inmedical therapy, symptomatic

    HF still confers a 20-25% riskof premature death in the first2.5 yrs after diagnosis. 50% of these premature

    deaths are SCD (VT/VF)1 Bardy G. The Sudden Cardiac Deatth-Heart Failure Trial

    (SCD-HeFT) in Woosley RL, Singh S, ArrhythmiaTreatment and Therapy, Copyright 2000 by MarcelDekker, Inc. , pp. 323-342,

    2 Sweeney MO PACE 2001;24:871-888.

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    SCA Relationship to HF

    Patients with HF are overall at 6-9 times higherrisk for SCD than general population

    As HF progresses, pump failure (rather than SCA)

    becomes relatively more likely as the cause ofdeath

    Reduced LVEF remains the single most important

    risk factor for overall mortality and suddencardiac death.

    Heart Disease and Stroke Statistics 2005 Update. AHA. www. americanheart.org

    S it f H t F il

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    Severity of Heart FailureModes of Death

    12%

    24%64%

    CHF

    Other

    SuddenDeath(N = 103)

    NYHA II

    26%

    15%59%

    CHF

    Other

    SuddenDeath

    (N = 103)

    NYHA III

    56%11%

    33%

    CHF

    Other

    SuddenDeath(N = 27)

    NYHA IV SCA Pump Failure

    NYHA Class II 64% 12%

    NYHA Class III 59% 26%

    NYHA Class IV 33% 56%

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    LVEF and SCA Incidence

    Vreede-Swagemakers JJ. J Am Coll Cardiol. 1997;30:1500-1505.

    LVEF

    0

    1

    2

    3

    4

    5

    6

    7

    8

    0-30% 31-40% 41-5 0% > 50%%

    SCAV

    ictims

    7.5%

    5.1%

    2.8%

    1.4%

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    SCD

    Genetic Basis

    Short QT syndrome

    Long QT Syndrome

    Brugada Syndrome

    Hypertrophic Cardiomyopathy

    Arrhythmogenic Right Ventricular Cardiomyopathy Catecholaminergic Polymorphic Ventricular

    Tachycardia

    Family history is a strong independent predictor ofsusceptibility to SCD

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    Schwartz PJ. Curr Probl Cardiol. 1997;22:297-351.Smith WM. Ann Intern Med. 1980;93:578-584.Garson A Jr. Circulation. 1993;87:1866-1872.

    Long QT Syndrome

    Congenital disorder that may lead tounexplained syncope, seizures, and SCA

    Either asymptomatic or are prone tosymptomatic and potentially lethal arrhythmias

    60% have a positive family history of LQTS orSCA

    Necessary to identify other family members atrisk

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    Long QT Syndrome in a 16-year-old girl

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    Myerburg RJ. Heart Disease, A Textbook of Cardiovascular Medicine. 5th ed, Vol 1.Philadelphia: WB Saunders Co; 1997:ch 24.Maron BJ. New Engl J Med. 2000;342:365-373.

    Hypertrophic Cardiomyopathy

    SCD is the most common cause of death

    Prevalence is about 0.2% of the general populationand about 10% of HCM patients are considered tobe at high risk of SCA

    Over a ten year period > 50% of high-risk patients

    would experience SCA

    Most common cause of SCA in athletes under age35

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    Arrhythmogenic RVDysplasia

    RV myocardium isprogressively replacedby fibrofatty tissue. LV isnormal

    LBBB morphology

    High risk of SCD ifarrhythmias are leftuncontrolled

    Risk stratification isimportant

    Tx: Drugs, ICD, ablation

    Prevent arrhythmicdeath influenced byautonomic tone

    Zipes and Jalife, 2001

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    Arrhythmogenic RV Cardiomyopathy

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    Brugada Syndrome

    ECG pattern of ST segmentelevation in V1 to V3 withRBBB morphology and

    syncopal or sudden deathepisodes in patients withstructurally normal hearts

    Male predominance

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    Brugada Syndrome

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    Brugada Syndrome Incidence

    Ubiquitous

    Cause of 4-12% of all sudden deaths

    Prevalence ?

    Pharmacologic maneuvers with Nablockers (Ajmaline, Flecainide,Procainamide) may unmask abnormalECG

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    Brugada Syndrome

    Etiology and Genetics

    SCN5A mutation

    Encodes Na channel

    Autosomal dominant pattern

    Males more prone to

    phenotype (typical ECG andventricular arrhythmias)

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    Brugada Syndrome

    Na channel blockers worsenECG findings

    Isoproterenol controls

    electrical storm Prognosis and Treatment:

    Poor if left untreated

    ICD: Treatment of choice

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    SUDS

    1997 Dr. KoonlaweeNademanee

    Thailand: Lai Tai Japan: Pokkuri

    Philippines: Bangungut

    Dying in their sleep

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    SUDS

    Clinical Presentation:Agonal respiration

    Unresponsiveness afterlabored respiration duringsleep or seizure-like

    symptomsNormal cardiac function

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    SUDS & Brugada

    No organic heart disease

    Males exclusively

    High incidence of induciblepolymorphic VT to VF

    High mortality rate

    Normalization of ECG on exercise EP mechanism same ??

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    Treatment Options for SCA

    Defibrillation is theonly effectivetreatment for SCA

    VF tends to rapidlydeteriorate intoasystole

    Asystole cannot besuccessfully treated

    with defibrillation

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    Small devices, pectoralimplant site

    Transvenous, single incision

    Local anesthesia; conscious sedation

    Short hospital stays

    Few complications

    Perioperative mortality < 1%

    Programmable therapy options

    Single- or dual-chamber therapy

    Battery longevity up to 9 years

    80,000 implants/year (2000 E)1

    Implantable Cardioverter Defibrillator

    First-line therapy for patients at risk for SCA

    1Morgan Stanley Dean Witter. Investors Guide to ICDs. 2000.

    Key Randomized Clinical

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    Key Randomized ClinicalTrials

    Adapted from: DiMarco JP. N Engl J Med. 2003;349:1836-47. www.medscape.com

    Young JB. Sudden cardiac death in heart failure. www.medscape.com

    ICD therapy for the secondary prevention of SCA

    Mortality

    (%)

    Trial N Age (yrs) Mean LVEF(%)

    Follow-up(mos)

    ControlTherapy

    Control ICD P

    AVID 1016 65 10 35 18 12 Amiodarone or sotalol

    24.0 15.8 .02

    CIDS 659 64 9 34 36 Amiodarone 29.6 25.3 .14

    CASH 288 58 11 45 57 34 Amiodarone or metoprolol

    44.4 36.4 .08

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    2006 ACC/AHA/ESC Guidelines for theManagement of Ventricular Arrhythmias:

    Secondary Prevention of SCD

    ICD Class I Recommendation: Patients with a history of SCA, VF,

    hemodynamically unstable VT, or unexplainedsyncope

    Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

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    Myerburg RJ, et al. Circulation. 1998. 97:1514-1521.

    Patients with a previous cardiac arrest are at high risk for subsequent SCA

    events but account for a small percentage of annual sudden deaths

    MADITI, MUSTT

    AVID, CASH,

    CIDS

    SCD-HeFT,MADIT II

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    Key Randomized Clinical Trials

    Adapted from: DiMarco JP. N Engl J Med. 2003;349:1836-47. www.medscape.com

    Kadish A, et.al. N Engl J Med2004;350:2151-8.

    Young JB. Sudden cardiac death in heart failure. www.medscape.com

    ICD therapy for the primary prevention of SCAMortality (%)

    Trial N Age(yrs)

    MeanLVEF (%)

    Follow-up(mos)

    ControlTherapy

    Control ICD P

    SCD-HeFT 2521 60.1 25 45.5 Optimal

    MedicalTherapy

    36.1 28.9 .007

    MADIT 196 63 9 26 27 Conventional 38.6 15.7 .009

    MADIT II 1232 64 10 23 20 OptimalMedicalTherapy

    19.8 14.2 .007

    MUSTT 704 67 12 30 39 No EP-guidedtherapy 48 24 .06

    DEFINITE 458 58 21 29.014.4 OptimalMedicalTherapy

    14.1 7.9 .08

    Heart Failure and Left Ventricular Dysfunction are

    http://www.medscape.com/http://www.medscape.com/
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    Myerburg RJ, et al. Circulation. 1998. 97:1514-1521.

    Heart Failure and Left Ventricular Dysfunction are

    indicators of SCA risk

    MADITI, MUSTT

    AVID, CASH,CIDS

    SCD-HeFT,MADIT II

    2006 ACC/AHA/ESC Guidelines for the

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    2006 ACC/AHA/ESC Guidelines for theManagement of Ventricular Arrhythmias: Primary

    Prevention of SCD

    ICD Class I Recommendations: Patients with ischemic cardiomyopathy who are at least 40

    days post-MI with an LVEF 30 - 40% and NYHA functionalclass II or III

    Patients with NYHA Class II-III, LVEF 30 - 35%, non-ischemiccardiomyopathy

    Patients who are at high risk of SCA due to genetic disorderssuch as long QT syndrome, Brugada syndrome, hypertrophiccardiomyopathy and arrhythmogenic right ventricular dysplasia(ARVD).

    Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

    2006 ACC/AHA/ESC Guidelines for the

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    2006 ACC/AHA/ESC Guidelines for theManagement of Ventricular Arrhythmias: Primary

    Prevention of SCD

    ICD Class II Recommendation: Ischemic and non-ischemic patients with NYHA functional

    class I, LVEF 30-35%

    Zipes, DP, et al. 2006 ACC/AHA/ESC Practice Guidelines 5. Circulation. 2006;114;385-484

    Many methods to further risk stratify patients

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    Many methods to further risk stratify patientsrisk for SCA have been studied...

    Test Objective Sensitivity

    (%)

    Specificity

    (%)

    Limitations

    Echo Measurement ofLVEF

    5565 7580

    HR

    variability

    Assessment of lowheart rate variability

    3862 7588 Multiple non-standardized methods

    EP Study Induction of VAs 4873 6593 Invasive, expensive

    Signal

    AveragedECG

    (SAECG)

    Induction of latepotentials

    56-68 7481 Not useful in non-ischemic cardiomyopathy

    Microvolt

    T-WaveAlternans(MTWA)

    Identification of

    repolarizationabnormalities

    7793 3783 Cannot be used in AF

    Siddiqui A, Kowey PR. Curr Opin Cardiol. 2006;21:517-25.

    Prior SG, et al. Eur Heart J, Vol 22:16:August 2001

    But a reduced EFremains the singlemost important riskfactor for overallmortality and suddencardiac death.

    E l ti P ti t t Ri k f SCA

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    Evaluating Patients at Risk for SCA

    Electrophysiology Studies (EPS) have been helpful

    in the diagnosis of cardiac arrhythmias including: Sinus and AV node dysfunction

    Conduction abnormalities

    Accessory pathways of conduction

    Inducibility of VT

    EPs can provide advanced treatments includingImplantable Cardioverter Defibrillators (ICDs) and

    ablation therapy

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    Conclusions

    The key to SCD prevention is to identifyhigh risk patients BEFORE they have aSCA event. The majority of cases are in

    patients with:

    Coronary artery disease, previous MI

    Low left ventricular ejection fraction

    Dilated cardiomyopathy and heart failure

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    INTERACTIVE SESSION

    Which is NOT a recognized risk factor

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    Which is NOT a recognized risk factorfor SCD?

    a. Short QT interval

    b. Brugada Syndrome

    c. Hypertrophic Cardiomyopathy

    d. Family History of SCD

    li i l t ti f SUDS

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    clinical presentation of SUDSexcept:

    a. agonal respiration

    b. unresponsiveness afterlabored respiration duringsleep or seizure-like

    symptomsc. Depressed cardiac function

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    Thank You