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Page 1: Sudden Cardiac Death

Sudden Cardiac DeathSudden Cardiac Death

Rosco GoreRosco Gore

Page 2: Sudden Cardiac Death

DefinitionDefinition

The natural death from cardiac The natural death from cardiac causes, heralded by abrupt loss of causes, heralded by abrupt loss of consciousness within 1 hour of the consciousness within 1 hour of the onset of an acute change in onset of an acute change in cardiovascular status. cardiovascular status.

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Epidemiology Epidemiology

300,000 cases per year300,000 cases per year 1-2/10001-2/1000 Bimodal distributionBimodal distribution

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CausesCauses

Long QT SyndromeLong QT Syndrome Brugada SyndromeBrugada Syndrome Hypertrophic Cardiomyopathy Hypertrophic Cardiomyopathy Arrhythmogenic Right Ventricular Arrhythmogenic Right Ventricular

Dysplasia Dysplasia Commotio Cordis Commotio Cordis Coronary AnomalyCoronary Anomaly

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Long QT SyndromeLong QT Syndrome

Autosomal Dominate- Romano-WardAutosomal Dominate- Romano-Ward Autosomal Recessive- Jervell and Autosomal Recessive- Jervell and

Lange-NielsenLange-Nielsen AcquiredAcquired 1/10,0001/10,000

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PresentationPresentation

Palpitations, Presyncope, Syncope, Palpitations, Presyncope, Syncope, Seizures, or Cardiac arrestSeizures, or Cardiac arrest

Asymptomatic prolonged QTcAsymptomatic prolonged QTc Referred by family membersReferred by family members Has a predilection for younger Has a predilection for younger

patientpatient

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QT intervalQT interval

Lead IILead II Include U wave if T/U wave merged and U Include U wave if T/U wave merged and U

wave >50% height of T wavewave >50% height of T wave Correct for rateCorrect for rate

Bazett formula =QT/(RR)^1/2Bazett formula =QT/(RR)^1/2 Fridericia’s cube-root =QT/(RR)^1/3Fridericia’s cube-root =QT/(RR)^1/3 Framingham Study Linear Regression Equation Framingham Study Linear Regression Equation

=QT + .154(1-RR) =QT + .154(1-RR) Average over 3-5 beatsAverage over 3-5 beats

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http://www.torsades.org/

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Diagnosis: Schwartz scoreDiagnosis: Schwartz score

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PrognosisPrognosis

Events increase w/ increasing QTcEvents increase w/ increasing QTc

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TherapyTherapy

NEJM 2008; 358:169-175NEJM 2008; 358:169-175

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Brugada SyndromeBrugada Syndrome

Autosomal dominate defect in Autosomal dominate defect in cardiac Na channels- variable cardiac Na channels- variable expressionexpression

0.4% US population0.4% US population Male predominanceMale predominance Average age of Dx=41Average age of Dx=41

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PathogenesisPathogenesis

Texas Heart Inst J 2007;34:67-75

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PresentationPresentation

Funny looking EGKsFunny looking EGKs SCD/Syncope SCD/Syncope TriggersTriggers

FeverFever SleepSleep Glucose/insulinGlucose/insulin Cocaine/ETOHCocaine/ETOH ElectrolytesElectrolytes

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DiagnosisDiagnosis

Type I ST pattern + one ofType I ST pattern + one of Documented ventricular fibrillation Documented ventricular fibrillation Self-terminating polymorphic ventricular Self-terminating polymorphic ventricular

tachycardia (VT) tachycardia (VT) Family history of sudden cardiac death at <45 years Family history of sudden cardiac death at <45 years Type 1 ST segment elevation in family members Type 1 ST segment elevation in family members Electrophysiologic inducibility of VT Electrophysiologic inducibility of VT Unexplained syncope suggestive of a Unexplained syncope suggestive of a

tachyarrhythmia tachyarrhythmia Nocturnal agonal respiration Nocturnal agonal respiration

Type II or III pattern + conversion to type I Type II or III pattern + conversion to type I +one of the above+one of the above

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PrognosisPrognosis

SCD or VFSCD or VF SyncopeSyncope Type I patternType I pattern

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TreatmentTreatment

ACC/AHA- AICDACC/AHA- AICD Class I- Pt w/ previous cardiac arrestClass I- Pt w/ previous cardiac arrest Class IIa- Pt w/ previous syncopeClass IIa- Pt w/ previous syncope Class IIa- Pt w/ previous VTClass IIa- Pt w/ previous VT

Quinidine and hydroguinidineQuinidine and hydroguinidine

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Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

AD defect in the myocardial AD defect in the myocardial contractile proteinscontractile proteins

Defective sarcomers->Myocyte Defective sarcomers->Myocyte dissaray->…Ventricular hypertrophydissaray->…Ventricular hypertrophy

0.16-0.29% US population0.16-0.29% US population

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PresentationPresentation

SCDSCD DOEDOE SyncopeSyncope Heart failureHeart failure Chest PainChest Pain PalpitationPalpitation AsymptomaticAsymptomatic

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Physical ExamPhysical Exam

Systolic Crescendo-decrescendo Systolic Crescendo-decrescendo murmur LLSB/apexmurmur LLSB/apex

Decreases w/ squatting, hand dripDecreases w/ squatting, hand drip Increase w/ standingIncrease w/ standing Bifid pulsesBifid pulses EKG-LVHEKG-LVH

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Ventricular ArrhythmiasVentricular Arrhythmias

Contributing factorsContributing factors Myocardial hypertrophyMyocardial hypertrophy Myocardial disarrayMyocardial disarray Myocardial fibrosisMyocardial fibrosis Myocardial ischemiaMyocardial ischemia Autonomic disturbanceAutonomic disturbance

SCDSCD 1% annual incidence1% annual incidence

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DiagnosisDiagnosis

ECHOECHO

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SCD RiskSCD Risk

High risk= 6%+High risk= 6%+ Low risk= 1% or Low risk= 1% or

<<

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TreatmentTreatment

PharmocologicPharmocologic VerapamilVerapamil Beta blockersBeta blockers DisopyramidDisopyramid

ICDICD Class I- h/o sustained VT/VFClass I- h/o sustained VT/VF One or more major risk factorOne or more major risk factor

Ablation/SurgeryAblation/Surgery

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PresentationPresentation

AsymptomaticAsymptomatic Palpitation, syncope, atypical chest Palpitation, syncope, atypical chest

pain, dyspneapain, dyspnea ArrhythmiasArrhythmias SCDSCD

Exercise associatedExercise associated

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DiagnosisDiagnosis

History Familial disease by histologyEKG Epsilon waves or QRS 110 ms+ in V1-V3

Biopsy Endomyocardial biopsy with fibrofatty replacementSevere isolated RV dilatation & dysfunction

Severe segmental dilatation of RVLocalized RV aneurysm (DK)

History Family history of SCDLBBB VT

Late potentials on SAECGTWI in V2 & V3 (no rbbb)

Mild isolated global RV dilatation and/or dysfunctionMild segmental dilatation of RV

Regional RV HKEcho/MRI

MINOR

Criteria for the Diagnosis of ARVD

MAJOR

Echo/MRI

EKG

2major or 1major + 2minor or 4minor

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TreatmentTreatment

ICD: ICD: Class I: History of sustained VT/VFClass I: History of sustained VT/VF Class IIa: extensive disease, LV Class IIa: extensive disease, LV

involvement, family members w/ SCD, involvement, family members w/ SCD, syncopesyncope

Amiodarone or Sotalol-Class IIAAmiodarone or Sotalol-Class IIA Ablation-Class IIAAblation-Class IIA

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Commotio Cordis Commotio Cordis

SCD due to low-impact precordial SCD due to low-impact precordial traumatrauma

Male predominance, young ageMale predominance, young age High mortalityHigh mortality Probability related to speed, time, Probability related to speed, time,

hardness of object and location of hardness of object and location of impactimpact

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Commotio cordisCommotio cordis

TreatmentTreatment CPRCPR ShockShock

PreventionPrevention Safety baseballsSafety baseballs Chest protectorsChest protectors

Follow upFollow up EKGEKG HolterHolter EchoEcho

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Congenital Coronary Artery Congenital Coronary Artery AnomaliesAnomalies

Variation in the take off the Coronary Variation in the take off the Coronary ArteriesArteries

Incidence 5.6Incidence 5.60.17%0.17% Deaths related to exertionDeaths related to exertion

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PresentationPresentation

AnginaAngina Atypical chest painAtypical chest pain SyncopeSyncope PalpitationPalpitation DizzinessDizziness SCDSCD

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PathophysiologyPathophysiology

Compression between the pulmonary Compression between the pulmonary artery and aortaartery and aorta

Acute angle take offAcute angle take off Myocardial necrosisMyocardial necrosis

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DiagnosisDiagnosis

ECHOECHO Cardiac MRICardiac MRI CT angiographyCT angiography Cardiac CathCardiac Cath

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Military SignificanceMilitary Significance

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TreatmentTreatment

Beta BlockersBeta Blockers SurgerySurgery Coronary StentsCoronary Stents Avoid strenuous activitiesAvoid strenuous activities

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What do you call 2 orthopedic surgeons reading an EKG?

A double blind study!!!!

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0/1-not advisable, 2/3 intermediate risk, 4/5-permitted

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0/1-not advisable, 2/3 intermediate risk, 4/5-permitted

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Athlete ScreeningAthlete Screening

ESC- EKGESC- EKG Italian experienceItalian experience

1979: 3.6/100K1979: 3.6/100K 2004: 0.4/100k2004: 0.4/100k

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A 32-year-old man is seen for an annual physical examination. There is no personal or family history of cardiovascular disease or symptoms. On physical examination, S1 and S2 are normal and there is an S4 present. There is a grade 2/6 crescendo-decrescendo systolic murmur heard best at the lower left sternal border. The murmur does not radiate to the carotid arteries. Valsalva maneuver increases the intensity of the murmur, and moving from a standing position to a squatting position, performing a passive leg lift while recumbent, and performing isometric handgrip exercises decreases the intensity. Rapid upstrokes of the peripheral pulses are present.

A Mitral valve prolapse

B Hypertrophic cardiomyopathy

C Atrial septal defect

D Ventricular septal defect

E Aortic stenosis

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A 32-year-old man is seen for an annual physical examination. There is no personal or family history of cardiovascular disease or symptoms. On physical examination, S1 and S2 are normal and there is an S4 present. There is a grade 2/6 crescendo-decrescendo systolic murmur heard best at the lower left sternal border. The murmur does not radiate to the carotid arteries. Valsalva maneuver increases the intensity of the murmur, and moving from a standing position to a squatting position, performing a passive leg lift while recumbent, and performing isometric handgrip exercises decreases the intensity. Rapid upstrokes of the peripheral pulses are present.

A Mitral valve prolapse

B Hypertrophic cardiomyopathy

C Atrial septal defect

D Ventricular septal defect

E Aortic stenosis

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A 23-year-old man is evaluated for palpitations that occur during exercise. He is otherwise healthy and takes no medications. Both the physical examination and the resting electrocardiogram are normal. A stress test demonstrates sustained monomorphic ventricular tachycardia at 201/min at peak exercise. There were no ischemic changes until the arrhythmia developed. The ventricular tachycardia had a left bundle and inferior axis morphology and terminated spontaneously after 7 minutes of rest. An echocardiogram is normal, and an MRI shows no abnormalities in the right or left ventricles.

What is the most likely etiology of ventricular tachycardia in this patient?

A Coronary spasm

B Idiopathic

C Arrhythmogenic right ventricular cardiomyopathy

D Infiltrative heart disease

E Anomalous origin of the coronary arteries

Page 65: Sudden Cardiac Death

A 23-year-old man is evaluated for palpitations that occur during exercise. He is otherwise healthy and takes no medications. Both the physical examination and the resting electrocardiogram are normal. A stress test demonstrates sustained monomorphic ventricular tachycardia at 201/min at peak exercise. There were no ischemic changes until the arrhythmia developed. The ventricular tachycardia had a left bundle and inferior axis morphology and terminated spontaneously after 7 minutes of rest. An echocardiogram is normal, and an MRI shows no abnormalities in the right or left ventricles.

What is the most likely etiology of ventricular tachycardia in this patient?

A Coronary spasm

B Idiopathic

C Arrhythmogenic right ventricular cardiomyopathy

D Infiltrative heart disease

E Anomalous origin of the coronary arteries

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A: ARVD

B: Goldwire’s space re-entry vehicle

C: Spaceballs hat hair