Sdd C di D thiSudden Cardiac Death in Children and ...

S dd C di D th iS dd C di D th iSudden Cardiac Death in Sudden Cardiac Death in Children and AdolescentsChildren and AdolescentsChildren and AdolescentsChildren and Adolescents

Kristopher C mbermack MDKristopher Cumbermack, MDAssistant Professor, Pediatrics

Contemporary PediatricsContemporary PediatricsMay 19, 2011

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Faculty DisclosureFaculty DisclosureFaculty DisclosureFaculty Disclosure• No disclosures or conflicts of interestNo disclosures or conflicts of interest

ObjectivesObjectivesObjectivesObjectives• Recognize the common causes, riskRecognize the common causes, risk

factors of sudden cardiac death in children

• Discuss the diagnostic tools available to identify potential risk factorsy p

• Review potential interventionsp

3

TopicsTopicsTopicsTopics• Sudden Cardiac DeathSudden Cardiac Death• Congenital Heart Disease

Arrhythmias• Arrhythmias• Cardiomyopathies• Prevention• Role of the Primary Care Physiciano e o e a y Ca e ys c a

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Sudden Death (SD)Sudden Death (SD)Sudden Death (SD)Sudden Death (SD)• Unexpected death in previously healthy• TraumaticMost common cause of SD in children~ 72 million in 1991: 21,367 SD (~30/100,000)

• Non-traumaticNon-cardiacSudden Cardiac Death (SCD) in childrenEstimates of 500/yr (0.8-6.2/100,000/yr)

5Rivara et al, Pediatrics 1996

Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac DeathSudden Cardiac Death• Abrupt (typically < 1 hr from event to death)Abrupt (typically 1 hr from event to death)• Unexpected

Cardiovascular etiology• Cardiovascular etiology• Absence of other fatal conditions

• Variety of mechanisms; often arrhythmiaa e y o ec a s s; o e a y a

6Zipes et al Circulation 1998

English Study on Sudden DeathEnglish Study on Sudden DeathEnglish Study on Sudden DeathEnglish Study on Sudden Death

Mean population of > 800 000 1 20 yr old for 10 years

7Wren et al, Heart 2000

Mean population of > 800,000 1-20 yr old for 10 years

Death in Young Competitive Athletes

Maron, B. J. et al. Circulation 2009;119:1085-1092 8



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Congenital Heart DiseaseCongenital Heart DiseaseCongenital Heart DiseaseCongenital Heart Disease• Repaired CHDRepaired CHDTetralogy of FallotTransposition of the great arteriesTransposition of the great arteriesSingle ventricle s/p Fontan procedure

• Unrepaired CHDAortic stenosisAortic stenosisCoronary artery anomalies

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Coronary Artery Origin Coronary Artery Origin AnomaliesAnomalies

• Left coronary artery from right aortic sinus• Right coronary artery from left aortic sinusg y y

• Incidence of 0 17 1 2%• Incidence of 0.17 – 1.2%

• 2nd most common cause of SCD in young athletes

11Davis et al, JACC 2001

Coronary Origin AnomaliesCoronary Origin AnomaliesCoronary Origin AnomaliesCoronary Origin Anomalies• Asymptomatic 70-80% y p

• Diagnostic testingNo physical exam findingsElectrocardiogram typically normalg yp yEchocardiographyEnsure reports comment on coronary originsEnsure reports comment on coronary origins

12Davis et al, JACC 2001

Left Coronary from Right SinusLeft Coronary from Right Sinusy gy gPosterior

ALCA

Anterior

Normal

Anterior

13Cheitlin et al, Herz 2009

NormalNormal

L ft C fL ft C fLeft Coronary from Left Coronary from Right SinusRight Sinus

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Right SinusRight SinusALCA

TreatmentTreatmentTreatmentTreatment

• Many recommend surgical interventionUnroofing procedureUnroofing procedure

C t d di t i l• Controversy depending on anatomical details due to limited data

15Frommelt et al, Pacing Clin EP, 2009

TopicsTopicsTopicsTopics• Sudden Cardiac Death OverviewSudden Cardiac Death Overview• Congenital Heart Disease


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Arrhythmias and SCDArrhythmias and SCDArrhythmias and SCDArrhythmias and SCD• Wolff-Parkinson-White SyndromeWolff Parkinson White Syndrome

• ChannelopathiespLong QT syndromeShort QT syndromeShort QT syndromeBrugada syndrome

• Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)Ventricular Tachycardia (CPVT)

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WolffWolff--Parkinson White Parkinson White Syndrome (WPWS)Syndrome (WPWS)

• Prevalence of 0 07% in children• Prevalence of 0.07% in children

• Supraventricular tachycardia (SVT)• Supraventricular tachycardia (SVT)Re-entrant (80%)Atrial fibrillation (15 30%) atrial flutter (5%)Atrial fibrillation (15-30%), atrial flutter (5%)

• Symptoms of SVT• Symptoms of SVT PalpitationsSyncopeSyncope

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WPW SyndromeWPW SyndromeWPW SyndromeWPW Syndrome

• Shortened PR interval

• Pre-excitation of the ventricle = delta waveventricle = delta wave

19www.rnceus.com/psvt/wpw.html

WPW PatternWPW PatternWPW PatternWPW Pattern• ECG pattern of pre-excitation onlyECG pattern of pre excitation only

• Asymptomatic without SVTy p

• Higher incidence than true WPW syndromeg y

• Theoretical risk for SCD

• Evaluation, intervention not defined

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WPW: ElectrophysiologyWPW: Electrophysiologyp y gyp y gyNormal Pre-excitation

21www.chrsonline.ca/patients/index.htm

WPW: Atrial Fibrillation/FlutterWPW: Atrial Fibrillation/Flutter

• Risk of SCD due to bypass of AV node

A fib/flutter

• Atrial tachycardia can conduct 1:1 to

VT/VFcan conduct 1:1 to ventricle via accessory pathwayaccessory pathway

22www.chrsonline.ca/patients/index.htm

TreatmentTreatmentTreatmentTreatment• Digoxin is contraindicatedDigoxin is contraindicated

• Beta blocker

• Class IC (flecainide)

• Electrophysiology study (EPS)Risk stratificationAblation

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Long QT Syndrome (LQTS)Long QT Syndrome (LQTS)Long QT Syndrome (LQTS)Long QT Syndrome (LQTS)• Prolongation of the QT intervalProlongation of the QT interval

• Genetic disorderMutations of ion channels affecting action

potentialpAutosomal dominantVariable expressionp

• Syncope, SCDy p ,

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Measuring the QT IntervalMeasuring the QT IntervalMeasuring the QT IntervalMeasuring the QT Interval

25Postema, Heart Rhythm 2008

LQTSLQTSLQTSLQTS

• General mechanism of syncope, SCDTriggerggVentricular tachycardia Torsades de pointesp

Inadequate O2 delivery to brain leads to syncopey pIf no return to sinus rhythm, SCD

26http://www.ncbi.nlm.nih.gov/books/NBK2219/

2008

al, L

ance

t M

orita

et a

M

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LQTS ChallengesLQTS Challenges

Hea

rt 20

01Vi

ncen

t, H

V

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LQTS ChallengesLQTS ChallengesLQTS ChallengesLQTS Challenges• Overlap of QT interval in normal and LQTSOverlap of QT interval in normal and LQTS

populations

• Secondary QT prolongation can occur mimicking LQTS or expose true LQTSg p

• Variable phenotype for same genotypep yp g yp

• 1st symptom may be SCDy y

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LQTS Treatment OptionsLQTS Treatment OptionsLQTS Treatment OptionsLQTS Treatment Options• Beta blockadeBeta blockadeMost effective in LQT1 with decreased

effectiveness in others

• Surgical denervation of the left cardiac gsympathetic nerve

• Automatic implantable cardioverter defibrillator (AICD)

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Suspect LQTS?Suspect LQTS?Suspect LQTS?Suspect LQTS?• Assessment of family history risk factors• Assessment of family history, risk factors

• Restrict from exercise• Restrict from exercise

• Urgent referral to pediatric cardiologist• Urgent referral to pediatric cardiologist

• If QTc > 500 ms consider empiricIf QTc > 500 ms, consider empiric treatment with beta blocker

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CommotioCommotio CordisCordis

010

l, N

EJM

2M

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et a

M

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CardiomyopathiesCardiomyopathiesCardiomyopathiesCardiomyopathies• Hypertrophic Cardiomyopathyyp p y p y• Dilated Cardiomyopathy• Restrictive Cardiomyopathy• Restrictive Cardiomyopathy• Myocarditis• Arrhythmogenic Right Ventricular

Cardiomyopathy/Dysplasia• Left ventricular non-compaction

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Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy

• Most common cause of SCD in young athletes in US

• Autosomal dominantAutosomal dominantVariety of sarcomere genes (>20 identified)Variable phenotypic expressionVariable phenotypic expressionMay not develop until adulthood

35


• Prevalence of 1 in 500 adults

• 1-20 yr old incidence of death in undiagnosed HCMundiagnosed HCM1 in 1.35 million/0.74 per million person yrs

36Wren et al Heart 2000


• Thickened ventricular walls Absence of other causes of hypertrophyWithout ventricular dilationSymmetric or asymmetric

• Systolic function typically maintained• Abnormal diastolic function (relaxation)( )

HCM: Mechanism of SCDHCM: Mechanism of SCDHCM: Mechanism of SCDHCM: Mechanism of SCD• Left ventricular outflow tract obstructionLeft ventricular outflow tract obstruction

• Disarray of myocardial muscle fibersy y

• Promotes arrhythmogenesisy g

• VT/VF thought to play significant role even g p y gw/o left ventricular outflow tract obstruction

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Athlete’s HeartAthlete’s HeartAthlete s HeartAthlete s Heart• Physiologic hypertrophyPhysiologic hypertrophy

• Exercise can cause cardiac remodelinggIncreased chamber sizeIncreased ventricular wall thicknessIncreased ventricular wall thickness

• Preserved systolic and diastolic functionese ed sys o c a d d as o c u c o

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36thB

ethhesda Confference: E

ligibility Reccom

mendatiions .., JAC

41

CC

2005

Dilated Dilated CardiomyopathyCardiomyopathyCardiomyopathyCardiomyopathy

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Left Ventricular Left Ventricular NonNon CompactionCompactionNonNon--CompactionCompaction

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Arrhythmogenic Right Arrhythmogenic Right Ventricular DysplasiaVentricular Dysplasia

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Our ChallengeOur ChallengeOur ChallengeOur Challenge• SCD is incredibly rare• SCD is incredibly rare

• First symptom is often SCD or aborted SCDFirst symptom is often SCD or aborted SCD

• Identify children at risk for SCD in feasibleIdentify children at risk for SCD in feasible and cost-effective manner

• Provide a reasonable intervention to decrease riskdecrease risk

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PreventionPreventionPreventionPrevention• Primary preventionPrimary preventionInterventions to decrease risk of initial SCD

• Secondary preventionI i d i k f bInterventions to decrease risk of subsequent

events

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PrePre--participation Screeningparticipation ScreeningPrePre participation Screeningparticipation Screening• Identify young athletes with 7Identify young athletes with

cardiovascular abnormalities

on 2

007

• Controversy regarding extent of screening of athletes C

ircul

atio

screening of athletesMedical history and examECG?

et a

l, C

ECG?Echocardiogram? M

aron

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Screening IssuesScreening IssuesScreening IssuesScreening Issues• Estimates of up to 10 million require

screening (high school > 5 million)

M t t ti ifi• Most asymptomatic or non-specific

C di l b liti 0 3% f• Cardiovascular abnormalities ~0.3% of athlete population

• Incidence of SCD in high school athletes 1/200 000~1/200,000 per year

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AHA Recommendations for AHA Recommendations for S i f C titi Athl tS i f C titi Athl tScreening of Competitive AthletesScreening of Competitive Athletes

• 12 key elements 712 key elementsPersonal HistoryFamily History on

200

7

Family HistoryPhysical Examination

Circ

ulat

io

• Parental verification important• Repeated after 2 years et

al,

C

• Repeated after 2 years• Repeated prior to college athletics M

aron

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Personal HistoryPersonal HistoryPersonal HistoryPersonal History• Exertional chest pain/discomfort 7Exertional chest pain/discomfort• Excessive exertional and unexplained

dyspnea/fatigue on 2

007

dyspnea/fatigue• Unexplained syncope/near syncope

f Circ

ulat

io

• Prior recognition of heart murmur• Elevated systemic blood pressure et

al,

CM

aron

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Family HistoryFamily Historyy yy y• Premature death before age of 50 yrs due to

h t di i ≥ 1 l ti 7heart disease in ≥ 1 relative

• Disability from heart disease in close relative on 2

007

• Disability from heart disease in close relative < 50 yrs

Circ

ulat

io

• Presence of specific cardiac conditions in family members et

al,

CM

aron

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Physical ExamPhysical ExamPhysical ExamPhysical Exam• Heart murmur 7Heart murmur

• Femoral pulses (coarctation) on 2

007

p ( )

• Physical stigmata of Marfan syndrome

Circ

ulat

io

y g y

• Brachial artery blood pressure et a

l, C

y p

Mar

on

53

• “At the discretion of the examiner,a positive response or finding in 20

07

a positive response or finding in any 1 or more of the 12 items may

cula

tion

be judged sufficient to trigger a referral for cardiovascular al

, Circ

evaluation.”

aron

et

Ma

Routine ECG Screening?Routine ECG Screening?Routine ECG Screening?Routine ECG Screening?• European Society of Cardiology and 7European Society of Cardiology and

International Olympic Committee recommend ECG screening on

200

7

recommend ECG screening• AHA does not endorse routine ECG;

remains at discretion of individual physician Circ

ulat

io

remains at discretion of individual physicianLow prevalenceLimited resources et

al,

C

Limited resourcesCosts ($2 billion/yr; $3.4 mill/prevented death)

Mar

on

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Exercise Restriction GuidelinesExercise Restriction GuidelinesExercise Restriction GuidelinesExercise Restriction Guidelines

• Detailed recommendations for conditions at risk for SCD

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Exercise Restriction Exercise Restriction GuidelinesGuidelines

57Mitchell et al, JACC 2005

Competitive Sport RestrictionCompetitive Sport RestrictionCompetitive Sport RestrictionCompetitive Sport Restriction

R t i ti f ll t• Restriction from all sportsCoronary artery origin anomalyHypertrophic cardiomyopathyUntreated WPW syndrome

• Restricted to Class IARestricted to Class IALong QT syndromeAICDAICD

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Primary PreventionPrimary Preventionyy• MedicationBeta blockade in LQT1

• AICDs/p congenital heart surgeryLQTSHCM

• EP study with ablationEP study with ablationWPW syndrome

• Surgical unroofing of anomalous coronary• Surgical unroofing of anomalous coronary59

Secondary PreventionSecondary PreventionSecondary PreventionSecondary Prevention• Competitive sports restrictionsCompetitive sports restrictions

• Medication

• AICD

• CPR training

• Automatic external defibrillators (AED)

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Automatic External Automatic External Defibrillator (AED)Defibrillator (AED)

• Ventricular fibrillation is common final pathway to SCD in many cases

• Time to defibrillation is most important factor for survival in out of hospital VF

• Survival < 10% if > 10 minutes

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AHA AED ProgramAHA AED ProgramAHA AED ProgramAHA AED Program• AED available within 5 minutesAED available within 5 minutesSchoolsOrganized SportsOrganized SportsTraining sitesArenas/StadiumsArenas/Stadiums

• CPR training for individuals involved at eachFi i i h• First activate emergency services, then initiate CPR and deploy AED

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Arrhythmias• Arrhythmias• Cardiomyopathies• Prevention• Role of the Primary Care Physiciano e o t e a y Ca e ys c a

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Role of the Primary Care Role of the Primary Care Ph i iPh i iPhysicianPhysician

• Clinical presentationClinical presentationExertional symptomsChest pain syncope dyspneaChest pain, syncope, dyspnea

Syncope Inconsistent w/ neurocardiogenic/vasovagal Inconsistent w/ neurocardiogenic/vasovagalOther etiology not found

Early fatigueEarly fatigue

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Role of the Primary Care Role of the Primary Care PhysicianPhysician

• Family HistoryRecurrent syncopeRecurrent syncope

Unexplained sudden death, SCD, SIDS

Known cardiovascular entities associated with SCDwith SCD

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• Physical ExamHarsh, systolic murmur at upper right sternal y pp g

border or click consistent w/ aortic stenosisValsalva maneuver or standing may

accentuate systolic murmur @ LLSB in HCMIrregular heart rateMarfan stigmata

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• High index of suspicion

If b i ECG• If concerns, obtain ECG

• Err on initial restriction of sports• Err on initial restriction of sportsUtilize 36th Bethesda recommendations

• Refer to pediatric cardiology for further evaluationevaluation

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AcknowledgementsAcknowledgementsAcknowledgementsAcknowledgements• Jason Grabham, RDCS, FASEJason Grabham, RDCS, FASE • Leslie Brashear, RDCS

Anil Attili MD• Anil Attili, MD• Douglas Schneider, MD• Thomas DiSessa, MD

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Q ti ?•Questions?

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update. Clin Res Cardiol, 2011. 100(5): p. 383-94. Antzelevitch, C., et al., Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the p y y yEuropean Heart Rhythm Association. Circulation, 2005. 111(5): p. 659-70.

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