-
Sudden CardiacSudden CardiacSudden Cardiac Sudden Cardiac
DeathDeath
Michelle A. Grenier, MDMichelle A. Grenier, MD,,Associate in
Pediatric CardiologyAssociate in Pediatric Cardiology
Texas Children’s HospitalTexas Children’s HospitalFinancial
Disclosures: None, but I do believe Prevention Financial
Disclosures: None, but I do believe Prevention
is a good thing.is a good thing.Unlabeled/Unapproved Uses
Disclosures: None.Unlabeled/Unapproved Uses Disclosures: None.
-
Athlete’s HeartAthlete’s HeartAthlete s HeartAthlete s Heart
•• HARVEY, La. HARVEY, La. ---- An autopsy An autopsy determined
that LSU baseball determined that LSU baseball player player Wally
PontiffWally Pontiff, found dead , found dead i hi h l ki hi h l
kin his parents' home last week, in his parents' home last week,
died of natural causes and no died of natural causes and no drugs
were in his system, the drugs were in his system, the Jefferson
Parish coroner saidJefferson Parish coroner saidJefferson Parish
coroner said Jefferson Parish coroner said Monday. The autopsy
found an Monday. The autopsy found an abnormality in the
heartabnormality in the heart, which , which is still being tested
so finalis still being tested so finalis still being tested, so
final is still being tested, so final determination of exact cause
of determination of exact cause of death may take several more
days, death may take several more days, the coroner's report
said.the coroner's report said.pp
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
Sudden cardiac death (SCD)Sudden cardiac death (SCD)Sudden
cardiac death (SCD)Sudden cardiac death (SCD)Nontraumatic,
nonviolent, Nontraumatic, nonviolent, unexpected event resulting
fromunexpected event resulting fromunexpected event resulting from
unexpected event resulting from sudden cardiac arrest within 6
sudden cardiac arrest within 6 hours of a previously witnessedhours
of a previously witnessedhours of a previously witnessed hours of a
previously witnessed state of normal health.state of normal
health.
Maron BJ, Epstein SE, Roberts WC. JACC 1986;7:204-14
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
Warning signs during exerciseWarning signs during exercise––
PalpitationsPalpitationspp–– DizzinessDizziness–– Chest Pain/ Chest
TightnessChest Pain/ Chest Tightness/ g/ g–– Shortness of
BreathShortness of Breath–– SyncopeSyncopeSyncopeSyncopeIf there
were warning signs, it may not be If there were warning signs, it
may not be
“Sudden”“Sudden”
-
How frequently does it occur?How frequently does it occur?How
frequently does it occur?How frequently does it occur?
l d h l ll d h l l•• Annual incidence in the general population
Annual incidence in the general population 1/100,000 all
comers1/100,000 all comers CDC, 2004, SenCDC, 2004, Sen--Chowdrhry,
McKenna, Chowdrhry, McKenna, Cardiology 2006Cardiology 2006gygy
•• 2.3/100,000 athletes per year2.3/100,000 athletes per year
Blangy, et al. Arch Mal Blangy, et al. Arch Mal Coeur Vaiss
2006Coeur Vaiss 2006
•• 4/100 000 hikers annually4/100 000 hikers annually B t h t l
I t J S t M dB t h t l I t J S t M d•• 4/100,000 hikers
annually4/100,000 hikers annually Burtscher, et al. Int J Sports
Med Burtscher, et al. Int J Sports Med 20072007
•• Incidence higher in athletes 2.5:1 Incidence higher in
athletes 2.5:1 Billes, et al. Eur J Billes, et al. Eur J C di P R h
bil 2006C di P R h bil 2006Cardiovasc Prev Rehabil 2006Cardiovasc
Prev Rehabil 2006
•• Incidence higher in males 9:1 Incidence higher in males 9:1
Billes et al. Eur J Cardiovasc Billes et al. Eur J Cardiovasc Prev
Rehabil 2006Prev Rehabil 2006
-
More Numbers…Looking at Athletes More Numbers…Looking at
Athletes ggunder 35 years of Ageunder 35 years of Age
•• >90% sudden death directly related to >90% sudden death
directly related to cardiovascular causescardiovascular causes
Bille, et al. Eur J Cardiovasc Prev Bille, et al. Eur J Cardiovasc
Prev Rehabil 2006Rehabil 2006
•• 50% congenital anatomical/cardiomyopathy50% congenital
anatomical/cardiomyopathy Bille, Bille, t l E J C di P R h bil
2006t l E J C di P R h bil 2006et al. Eur J Cardiovasc Prev Rehabil
2006et al. Eur J Cardiovasc Prev Rehabil 2006
•• 10% early atherosclerotic disease10% early atherosclerotic
disease Bille, et al. Eur J Bille, et al. Eur J Cardiovasc Prev
Rehabil 2006Cardiovasc Prev Rehabil 2006Cardiovasc Prev Rehabil
2006Cardiovasc Prev Rehabil 2006
•• 4% 164% 16--64 years no cause ID: arrhythmia? 64 years no
cause ID: arrhythmia? SenSen--Chowdhry McKenna Cardiol 2006Chowdhry
McKenna Cardiol 2006Chowdhry, McKenna Cardiol 2006Chowdhry, McKenna
Cardiol 2006
-
The Invincible AdolescentThe Invincible AdolescentThe Invincible
AdolescentThe Invincible Adolescent
•• 40% occurred in athletes
-
What is the relative cost of SCD?What is the relative cost of
SCD?What is the relative cost of SCD?What is the relative cost of
SCD?
•• “The term ‘sudden cardiac death’ (SCD) “The term ‘sudden
cardiac death’ (SCD) implies the sudden and unexpected loss of
implies the sudden and unexpected loss of a productive member of
the community.” a productive member of the community.” Smith and
Cain. J Interv Card Electrophysiol 2007Smith and Cain. J Interv
Card Electrophysiol 2007
•• “The cost of SCD to society is incalcuable.” “The cost of SCD
to society is incalcuable.” Smith and Cain. J Interv Card
Electrophysiol 2007Smith and Cain. J Interv Card Electrophysiol
2007
If it i f il b it ff tIf it i f il b it ff t•• If it is your
family member, it affects you If it is your family member, it
affects you 100%.100%.
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
k f l f dd dk f l f dd dRisk profile for sudden cardiac Risk
profile for sudden cardiac deathdeath
17 di17 di•• 17 years median age 17 years median age •• 90%
male90% male•• 52% white, 44% black52% white, 44% black
68% l i f tb ll b k tb ll68% l i f tb ll b k tb ll•• 68% playing
football or basketball68% playing football or basketball•• 90%
collapsed during or shortly 90% collapsed during or shortly
after training session/competitive after training
session/competitive eventeventeventevent
•• 85% cardiovascular cause85% cardiovascular cause
Maron BJ et al. JAMA 1996; 276: 199-204
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
Incidence of Sudden Cardiac Death by Sport
http://www.suddendeathathletes.org
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
Incidence of Sudden Cardiac Death by Month
http://www.suddendeathathletes.org
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
Incidence of Sudden Cardiac Death by Time of Day
http://www.suddendeathathletes.org
-
Sudden cardiac death in Sudden cardiac death in
AthletesAthletesDistribution of cardiovascular causes of sudden
death in 1435 young competitive athletes
Maron, B. J. et al. Circulation 2007;115:1643-1455
-
Athlete’s HeartAthlete’s HeartAthlete s HeartAthlete s Heart••
At age 18, just one At age 18, just one g , jg , j
month shy of his high month shy of his high school graduation,
school graduation, Lil VicLil Vicwent into sudden cardiacwent into
sudden cardiacwent into sudden cardiac went into sudden cardiac
arrest while doing what arrest while doing what he loved best he
loved best -- playing playing b k b ll db k b ll dbasketball. It
turned out basketball. It turned out that Vic had that Vic had
hypertrophic hypertrophic yp pyp pcardiomyopathy cardiomyopathy
(HCM)(HCM) or an enlarged or an enlarged heart which had beenheart
which had beenheart, which had been heart, which had been
undetected. undetected.
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
Hypertrophic Cardiomyopathy
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Etiologies of SCDEtiologies of SCD–– Hypertrophic
CardiomyopathyHypertrophic Cardiomyopathy
••Leading cause of sudden Leading cause of sudden ead g cause o
suddeead g cause o suddecardiac death in young cardiac death in
young athletesathletes
••Estimated prevalence of the Estimated prevalence of the
disease 1:500 (not all high disease 1:500 (not all high risk)
risk)
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• HypertrophicHypertrophic•• Hypertrophic Hypertrophic
CardiomyopathyCardiomyopathy
Disorder caused byDisorder caused by––Disorder caused by
Disorder caused by mutations of the cardiac mutations of the
cardiac sarcomere unitsarcomere unitsarcomere unitsarcomere
unit
––Autosomal dominant Autosomal dominant
hhinheritanceinheritance
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Detection of HCMDetection of HCM–– HistoryHistory
••F il hi tF il hi t••Family historyFamily history––autosomal
dominantautosomal dominant
••SymptomsSymptomsy py p––exertional dyspnea, orthopnea,
exertional dyspnea, orthopnea,
fatigue: diastolic dysfunction fatigue: diastolic dysfunction
––chest pain: ischemiachest pain: ischemiachest pain: ischemiachest
pain: ischemia
••ExamExam––murmur murmur (present in only 25%)(present in only
25%)
-
SCD In Young AthletesSCD In Young AthletesSCD In Young
AthletesSCD In Young Athletes
Hypertrophic cardiomyopathyHypertrophic cardiomyopathy
No murmur on exam Possible murmur on exam
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
i f Ci f C•• Detection of HCMDetection of HCM–– MurmurMurmur
••SEM at the left lower to mid sternalSEM at the left lower to
mid sternal••SEM at the left lower to mid sternal SEM at the left
lower to mid sternal edge, radiates along the LVOTedge, radiates
along the LVOT
••Harsh in quality, but may be vibratoryHarsh in quality, but
may be vibratory••Increases with exercise Increases with
exercise
––increased contractilityincreased contractilityI ith V l l t
diI ith V l l t di••Increases with Valsalva, standing Increases
with Valsalva, standing
––decreased volumedecreased volume
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Detection of HCM: Detection of HCM: Inherent
difficultiesInherent difficulties–– Children may not yet show
Children may not yet show
LVHLVHLVH tLVH t–– LVH may appear at any LVH may appear at any
age, and change.age, and change.
–– It may not appear untilIt may not appear untilIt may not
appear until It may not appear until adolescence, as the normal
adolescence, as the normal LV remodeling process LV remodeling
process appears to be most activeappears to be most activeappears
to be most active appears to be most active thenthen
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Sudden Death in HCMSudden Death in HCM–– Lethal ventricular
arrhythmiaLethal ventricular arrhythmia–– May be associated with
extreme May be associated with extreme
physical exertion physical exertion M l b i it t d bM l b i it t
d b–– May also be precipitated by May also be precipitated by
volume depletion (dehydration).volume depletion (dehydration).
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Risk Factors for SCD in HCMRisk Factors for SCD in HCM––
Prior cardiac arrestPrior cardiac arrest–– Sustained or repetitive
non sustained VT Sustained or repetitive non sustained VT ––
Hypotensive BP response to exerciseHypotensive BP response to
exercise
F il hi t f SCDF il hi t f SCD–– Family history of SCDFamily
history of SCD–– Syncope (particularly exrtional)Syncope
(particularly exrtional)–– Extreme LVH (>30mm)Extreme LVH
(>30mm)Extreme LVH (>30mm)Extreme LVH (>30mm)
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
i f SC i Ci f SC i C•• Prevention of SCD in HCMPrevention of SCD
in HCM–– Medications have not been Medications have not been
h t ff t th t ff t tshown to affect outcomeshown to affect
outcome–– One study from Italy, where One study from Italy,
where
th i ti id ith i ti id ithere is nationwide screening, there is
nationwide screening, showed a diminished incidence showed a
diminished incidence in SCD (ARVD), with exercisein SCD (ARVD),
with exercisein SCD (ARVD), with exercise in SCD (ARVD), with
exercise restrictionrestriction
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
•• Sudden Death in HCMSudden Death in HCML th l h t h th b litL
th l h t h th b lit–– Lethal heart rhythm abnormalityLethal heart
rhythm abnormality
–– Association with extreme physical Association with extreme
physical exertionexertionexertion exertion
–– Autosomal Dominant/Spontaneous Autosomal Dominant/Spontaneous
mutationsmutations
–– Warrants disqualification from Warrants disqualification from
competitive sports competitive sports
-
HCM versus Athlete’s HeartHCM versus Athlete’s HeartHCM versus
Athlete s HeartHCM versus Athlete s Heart
•• Pathologic descriptions of myocardial Pathologic descriptions
of myocardial hypertrophy in trained individuals from hypertrophy
in trained individuals from Ki h d Li b h i 1958Ki h d Li b h i
1958Kirch and Linzbach in 1958Kirch and Linzbach in 1958
•• The heart of the trained athlete can be The heart of the
trained athlete can be t i th l i b t hi t l it i th l i b t hi t l
itwice the normal size, but histologic twice the normal size, but
histologic structure remains intactstructure remains intact
•• Th i ht f th t i d h t d tTh i ht f th t i d h t d t•• The
weight of the trained heart does not The weight of the trained
heart does not usually surpass the limit of 500g, defined usually
surpass the limit of 500g, defined as the critical heart weightas
the critical heart weightas the critical heart weightas the
critical heart weight
-
Athlete’s HeartAthlete’s HeartAthlete s HeartAthlete s Heart••
Cardiac enlargement in athletes may Cardiac enlargement in athletes
may
represent a physiologic phenomenon represent a physiologic
phenomenon (controversial for many years)(controversial for many
years)
•• Concerns about longConcerns about long--term consequences
term consequences of morphologic left ventricular remodelingof
morphologic left ventricular remodelingp g gp g g
•• Concern of sudden cardiac deaths in Concern of sudden cardiac
deaths in athletes with hypertrophic cardiomyopathyathletes with
hypertrophic cardiomyopathyathletes with hypertrophic
cardiomyopathy athletes with hypertrophic cardiomyopathy (HCM)
accounting for majority of (HCM) accounting for majority of
catastrophescatastrophescatastrophescatastrophes
-
Ambiguity with HCMAmbiguity with HCMAmbiguity with HCMAmbiguity
with HCM
Figure 1. Echocardiograms in parasternal long-axis view from an
elite athlete (Olympic rower) (left) and a young asymptomatic
patient with hypertrophic cardiomyopathy (HCM) (right). Magnitude
of anterior ventricular septal (VS) hypertrophy is similar in each,
demonstrating the morphological gray zone into which a highly
trained athlete may fall and the diagnostic ambiguity that may
ensue. Calibration dots are 1 cm apart. Leftdiagnostic ambiguity
that may ensue. Calibration dots are 1 cm apart. Left panel is
reprinted by permission of the New England Journal of
Medicine(1991;324:295-301).
-
Differentiating from HCMDifferentiating from HCMDifferentiating
from HCMDifferentiating from HCM•• LV cavity enlarged in athlete’s
heartLV cavity enlarged in athlete’s heartLV cavity enlarged in
athlete s heartLV cavity enlarged in athlete s heart•• Hypertrophy
symmetric and homogenousHypertrophy symmetric and homogenous•• LV
cavity maintains ellipsoid shapeLV cavity maintains ellipsoid
shape•• LV cavity maintains ellipsoid shapeLV cavity maintains
ellipsoid shape•• LV mass regresses with deconditioningLV mass
regresses with deconditioning•• LV filling pattern remains normalLV
filling pattern remains normal•• Acoustic pattern of myocardium
remainsAcoustic pattern of myocardium remainsAcoustic pattern of
myocardium remains Acoustic pattern of myocardium remains
normalnormal•• Restricted to certain sportsRestricted to certain
sports•• Restricted to certain sportsRestricted to certain
sports
-
Cardiac FunctionCardiac FunctionCardiac FunctionCardiac
Function•• Echo exams on 286 cyclists in Tour de Echo exams on 286
cyclists in Tour de yy
France and 52 matched sedentary France and 52 matched sedentary
volunteers volunteers
•• 148 in 1995 race, 138 in 1998 race, and 148 in 1995 race, 138
in 1998 race, and 37 in both37 in both37 in both37 in both
•• 51% had substantial LV enlargement 51% had substantial LV
enlargement (LVIDd > 60mm up to 73mm)(LVIDd > 60mm up to
73mm)(LVIDd > 60mm, up to 73mm)(LVIDd > 60mm, up to 73mm)
•• 9% had increased LVWT (>13 mm)9% had increased LVWT
(>13 mm)
-
A Heavy HeartA Heavy HeartA Heavy HeartA Heavy Heart
•• Left Ventricular Mass (PennLeft Ventricular Mass (Penn--cube
formula)cube formula)
ControlsControls 174 g174 gEnduranceEndurance trainedtrained 249
g249 gEnduranceEndurance--trainedtrained 249 g249 gCombinedCombined
288 g288 gStrengthStrength--trainedtrained 267 g267 g
-
A Summary Table to ClarifyA Summary Table to ClarifyA Summary
Table to Clarify A Summary Table to Clarify
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
Commotio cordisCommotio cordisCommotio cordisCommotio
cordis••Direct, nonpenetrating Direct, nonpenetrating trauma to the
chest wall trauma to the chest wall t au a to t e c est at au a to
t e c est a––baseball, hockey puck, baseball, hockey puck,
opponentopponent
O d i l i iO d i l i i••Occurs during repolarizationOccurs
during repolarization––electrically vulnerableelectrically
vulnerable
••10% survival10% survival••10% survival10% survival••Softer
baseballs shown to Softer baseballs shown to reduce riskreduce
riskreduce riskreduce risk
-
SCD In Young AthletesSCD In Young AthletesSCD In Young
AthletesSCD In Young Athletes
Commotio Cordis (20%)Commotio Cordis (20%)
http://www.suddendeathathletes.org
-
SCD In Young AthletesSCD In Young AthletesSCD In Young
AthletesSCD In Young Athletes
C ti C di V l bl i d iC ti C di V l bl i d iCommotio Cordis:
Vulnerable microseconds in Commotio Cordis: Vulnerable microseconds
in the cardiac cyclethe cardiac cycle
Ventricular Fibrillation
-
SCD in Young AthletesSCD in Young Athletesgg–– Long QT
SyndromeLong QT Syndrome
C it l i d fC it l i d f••Congenital, acquired formsCongenital,
acquired forms••Classically defined by:Classically defined by:
QT 440QT 440––QTc > 440 msecQTc > 440 msec––Bizarre T wave
morphologyBizarre T wave morphologyT ltT lt––T wave alternansT wave
alternans
––Predisposition to lethal cardiac Predisposition to lethal
cardiac arrhythmias (arrhythmias (torsade de pointes)torsade de
pointes)arrhythmias (arrhythmias (torsade de pointes)torsade de
pointes)
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Long QT syndrome and SCDLong QT
syndrome and SCDg Q yg Q y
–– 287 patients287 patients••Mean age at death 6.8 +/Mean age at
death 6.8 +/-- 5.6 yrs5.6 yrs
W i iW i i••Warning signs:Warning signs:––syncope (26%),
seizures (10%), syncope (26%), seizures (10%),
cardiac arrest (9%) preceded bycardiac arrest (9%) preceded
bycardiac arrest (9%) preceded by cardiac arrest (9%) preceded by
emotion or exerciseemotion or exercise
••Family historyFamily history39% f Q S 3 % f SC39% f Q S 3 % f
SC––39% positive for LQTS, 31% for SCD39% positive for LQTS, 31%
for SCD
Garson et al. Circulation 1993; 87(6): 1866-72
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
•• Coronary Artery AnomaliesCoronary Artery Anomalies–– Second
leading cause of SCDSecond leading cause of SCDSecond leading cause
of SCDSecond leading cause of SCD–– Mechanism of sudden
deathMechanism of sudden death
•• Abrupt lack of oxygen to the heart muscleAbrupt lack of
oxygen to the heart muscle•• Heart rhythm abnormalityHeart rhythm
abnormality
–– No known inheritance patternNo known inheritance pattern
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
Coronary Artery Abnormalities (14%)Coronary Artery Abnormalities
(14%)
The right coronary artery arises from the right cusp, the left
from the left cusp
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
Acute angle takeoff and course between the aortaAcute angle
takeoff and course between the aorta and pulmonary artery. Both
exacerbated by y
aortic root dilation with exercise.
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Coronary ArteryCoronary
ArteryCoronary Artery Coronary Artery
AnomaliesAnomalies••Second leading disease of the Second leading
disease of the gg
heart resulting in SCDheart resulting in SCD••Mechanism of
sudden death: Mechanism of sudden death:
lethal ventricular arrhythmialethal ventricular arrhythmialethal
ventricular arrhythmialethal ventricular arrhythmia––sudden onset
critical sudden onset critical
ischemiaischemiat i f tit i f ti––recurrent infarctionsrecurrent
infarctions
––neurocardiogenic syncopeneurocardiogenic syncope
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Marfan SyndromeMarfan Syndrome–– Incidence of SCD 5Incidence
of SCD 5--8/10008/1000–– Ruptured aortic aneurysmRuptured aortic
aneurysm
Disproportionate representationDisproportionate representation––
Disproportionate representation Disproportionate representation
among basketball & volleyball players among basketball &
volleyball players (Flo Hyman)(Flo Hyman)
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
M f S dMarfan Syndrome
Long, spider-like fingers
Photos courtesy of Harold Chen, MD. Emedicine: Marfan
Syndrome
Tall stature, long arms Hyperflexible joints
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes–– Marfan SyndromeMarfan Syndrome
A l d iA l d i••Autosomal dominantAutosomal dominant••Prevalence
1:10,000Prevalence 1:10,000••Defect in the fibrillin geneDefect in
the fibrillin gene••Defect in the fibrillin geneDefect in the
fibrillin gene••Tall habitus, arachnodactyly, Tall habitus,
arachnodactyly,
pectus excavatum, high arched pectus excavatum, high arched p ,
gp , gpalate, pes planus, scoliosis, palate, pes planus, scoliosis,
ectopia lentis, hyperextensibiliity, ectopia lentis,
hyperextensibiliity, dilated aortic root, mitral valvedilated
aortic root, mitral valvedilated aortic root, mitral valve dilated
aortic root, mitral valve prolapseprolapse
••LoeysLoeys--Dietz: TGF b abnormalityDietz: TGF b
abnormality
-
Sudden Cardiac DeathSudden Cardiac Death––
MyocarditisMyocarditis
••Viral infection of the heart muscleViral infection of the
heart muscle••Heritable predispositionHeritable predisposition
–– Long QT syndromeLong QT syndrome••Abnormality of the
electrical systemAbnormality of the electrical system••60%
heritable (may be higher)60% heritable (may be higher)
A ti t iA ti t i–– Aortic stenosisAortic stenosis••Birth defect
of the heart resulting in Birth defect of the heart resulting
in
progressive obstruction of blood flow fromprogressive
obstruction of blood flow fromprogressive obstruction of blood flow
from progressive obstruction of blood flow from the heart to the
bodythe heart to the body
••May be inherited 30May be inherited 30--50%, NODAL and 50%,
NODAL and ay b d 30ay b d 30 50%, O a d50%, O a dNOTCH 1NOTCH 1
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
OC SOC S••MYOCARDITISMYOCARDITIS••1818--29% of childhood SCD29%
of childhood SCDI fl ti di tI fl ti di t••Inflammation predisposes
to Inflammation predisposes to arrhythmia up to months
laterarrhythmia up to months later
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
•• Arrhythmogenic right ventricular Arrhythmogenic right
ventricular cardiomyopathycardiomyopathy
••F tt i filt ti d fib i f th i htF tt i filt ti d fib i f th i
ht••Fatty infiltration and fibrosis of the right Fatty infiltration
and fibrosis of the right ventricleventricle
••Predisposed to exercisePredisposed to exercise--induced
induced ppventricular arrhythmiasventricular arrhythmias
••Genetic basisGenetic basis
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Conduction AbnormalitiesConduction
Abnormalities•• Conduction AbnormalitiesConduction
Abnormalities
••WolffWolff--ParkinsonParkinson--White syndromeWhite
syndrome––Ventricular preVentricular
pre--excitationexcitationVentricular preVentricular pre
excitationexcitation––Supraventricular tachycardiaSupraventricular
tachycardiaincidence same in athletes andincidence same in athletes
andincidence same in athletes and incidence same in athletes and
nonnon--athletesathletes
––Sudden deathSudden deathSudden deathSudden deathatrial
fibrillation with rapid atrial fibrillation with rapid conduction
along the bypass conduction along the bypass g ypg yptract
(VF)tract (VF)
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Miscellaneous Causes of
SCDMiscellaneous Causes of SCDs a ous Caus s o SCs a ous Caus s o
SC
–– Cocaine/OtherCocaine/OtherRx, Steroids, “Huffing”Rx,
Steroids, “Huffing”, , g, , g–– Atherosclerotic coronary artery
Atherosclerotic coronary artery
diseasedisease22 3% f SCD i thl t 403% f SCD i thl t 40••22--3%
of SCD in athletes < 40 years3% of SCD in athletes < 40
years
••Is this on the rise?Is this on the rise?Ano e i ne o /b
lemiAno e i ne o /b lemi–– Anorexia nervosa/bulemiaAnorexia
nervosa/bulemia••electrolyte imbalances, conduction electrolyte
imbalances, conduction
abnormalitiesabnormalitiesabnormalitiesabnormalities
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes
How do we How do we costcost--effectivelyeffectivelycostcost
effectively effectively
reduce the reduce the i id fi id fincidence of incidence of
sudden death sudden death sudde deatsudde deatin our athletes?in
our athletes?
-
Primary PreventionPrimary PreventionPrimary Prevention Primary
Prevention •• Do not let anyone Do not let anyone
participate inparticipate inparticipate in participate in
sportssports
-
Primary PreventionPrimary PreventionPrimary PreventionPrimary
Prevention
SS•• PrePre--participation Screeningparticipation Screening––
Padua, Italy:Padua, Italy: Nationwide systematic athletic
Nationwide systematic athletic
screening implemented in 1982 (ages 12screening implemented in
1982 (ages 12 –– 3535screening implemented in 1982 (ages 12
screening implemented in 1982 (ages 12 35 35 years)years)
–– There was a There was a significant reductionsignificant
reduction of SCD in of SCD in this population (0 56 vs 0 21)this
population (0 56 vs 0 21)this population (0.56 vs. 0.21)this
population (0.56 vs. 0.21)
–– The causes of SCD differ in Italy (ARVD, HCM)The causes of
SCD differ in Italy (ARVD, HCM)–– Stringent
disqualificationStringent disqualificationStringent
disqualificationStringent disqualification–– The Lausanne
RecommendationsThe Lausanne Recommendations
(International Olympic Committee)(International Olympic
Committee)f d lf d l–– European Society of Cardiology Consensus
European Society of Cardiology Consensus
StatementStatement
-
What is the US Consensus?What is the US Consensus?What is the US
Consensus?What is the US Consensus?
•• 19961996, the , the American Heart Association American Heart
Association consensus panelconsensus panel recommended
prerecommended pre--participation cardiovascular screening for
participation cardiovascular screening for young, competitive
athletes based on young, competitive athletes based on “ethical,
legal and medical grounds”.“ethical, legal and medical
grounds”.
•• The Bethesda Conference The Bethesda Conference JACC 2005JACC
2005
-
Screening athletesScreening athletesScreening athletesScreening
athletes
•• AHAAHA recommends “a complete and recommends “a complete and
targeted personal and family history and targeted personal and
family history and physical examination (including brachial
physical examination (including brachial artery blood pressure
measurement) artery blood pressure measurement) performed by
performed by a qualified examinera qualified examiner and and
include include 12 key AHA12 key AHA--recommended recommended
elementselements as well as parental verification of as well as
parental verification of medical history for high school and middle
medical history for high school and middle school athletes”school
athletes”
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes
•• The 12 Element AHA Recommendations The 12 Element AHA
Recommendations for Preparticipation CV Screening of for
Preparticipation CV Screening of C titi Athl t (2006C titi Athl t
(2006 7)7)Competitive Athletes (2006Competitive Athletes
(2006--7)7)
•• Medical HistoryMedical History•• Personal HistoryPersonal
History
–– Exertional chest pain/DiscomfortExertional chest
pain/Discomfort–– Unexplained Syncope/Near syncopeUnexplained
Syncope/Near syncope–– Prior recognition of heart murmurPrior
recognition of heart murmur
l d bl dl d bl d–– Elevated systemic blood pressureElevated
systemic blood pressure
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes
–– Is there Family History?Is there Family History?•• Premature
deathPremature death (sudden, and unexpected, or (sudden, and
unexpected, or
otherwise) before age 50 years due to heartotherwise) before age
50 years due to heartotherwise) before age 50 years due to heart
otherwise) before age 50 years due to heart disease, in > 1
relativedisease, in > 1 relative
•• Disability from heart diseaseDisability from heart disease in
a close relative in a close relative < 50 years of age< 50
years of age< 50 years of age< 50 years of age
•• Specific knowledge of Specific knowledge of certain cardiac
certain cardiac conditionsconditions in family members:
hypertrophic or in family members: hypertrophic or d l d d h l dd l
d d h l ddilated cardiomyopathy, long QT syndrome or dilated
cardiomyopathy, long QT syndrome or other ion channelopathies,
Marfan syndrome, or other ion channelopathies, Marfan syndrome, or
clinically important arrhythmiasclinically important
arrhythmias
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes•• Physical ExamPhysical Examyy
––Heart murmurHeart murmurF l l l d iF l l l d i––Femoral pulses
to exclude aortic Femoral pulses to exclude aortic
coarctationcoarctation
––Physical stigmata of Marfan Physical stigmata of Marfan
syndromesyndromesyndromesyndrome
––Brachial artery blood pressure Brachial artery blood pressure
(sitting position)(sitting position)
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes•• NBANBA requires echo and ECG on all requires echo and
ECG on all qq
players on an annual basisplayers on an annual basis•• NFLNFL
teams generally perform ECG’steams generally perform ECG’s•• NFL
NFL teams generally perform ECG s teams generally perform ECG s
and obtain echos if clinically indicatedand obtain echos if
clinically indicated•• US Olympic CommitteeUS Olympic Committee
administers preparticipation H & Padministers
preparticipation H & Padministers preparticipation H & P
administers preparticipation H & P and further noninvasive
testing is and further noninvasive testing is performed only when
warrantedperformed only when warrantedperformed only when
warrantedperformed only when warranted
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes•• International Olympic Committee andInternational Olympic
Committee andInternational Olympic Committee and International
Olympic Committee and
European Society of CardiologyEuropean Society of
Cardiologyrecommend ECG’s in all young competitiverecommend ECG’s
in all young competitiverecommend ECG s in all young competitive
recommend ECG s in all young competitive athletesathletes
•• AHA panelAHA panel does not believe it to be eitherdoes not
believe it to be either•• AHA panelAHA panel does not believe it to
be either does not believe it to be either prudent or practical to
recommend the prudent or practical to recommend the routine use of
tests such as 12routine use of tests such as 12--lead ECGlead
ECGroutine use of tests such as 12routine use of tests such as 12
lead ECG lead ECG or echocardiography in the context of or
echocardiography in the context of mass universal screeningmass
universal screeningmass, universal screeningmass, universal
screening
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes•• More than More than 5 million5 million competitive
competitive pp
athletes in high schoolathletes in high school•• Greater
thanGreater than 500 000500 000 collegiatecollegiate•• Greater than
Greater than 500,000500,000 collegiate collegiate
athletes athletes •• Greater than Greater than 5,0005,000
professional professional
athletesathletesathletesathletes•• Population available for
screening Population available for screening
may be as large asmay be as large as 10 million10 millionmay be
as large as may be as large as 10 million10 million
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes•• ECGECG abnormal in > 90% HCMabnormal in > 90%
HCM•• Detects ARVC, ion channelopathiesDetects ARVC, ion
channelopathies
Mi h l i i l hiMi h l i i l hi•• Misses catecholaminergic
polymorphic Misses catecholaminergic polymorphic ventricular
tachycardiaventricular tachycardia
•• ECGECG has low specificity as a has low specificity as a
screening test in athletic populationsscreening test in athletic
populationsscreening test in athletic populationsscreening test in
athletic populations
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Concerns re: widespread use of
Concerns re: widespread use of
i i t tii i t tinoninvasive testingnoninvasive
testing––FalseFalse--positive test resultspositive test results
••unnecessary anxietyunnecessary anxiety••unjustified exclusion
fromunjustified exclusion fromunjustified exclusion from
unjustified exclusion from sportssports
••unjustified exclusion fromunjustified exclusion
fromunjustified exclusion from unjustified exclusion from life
insurance coveragelife insurance coverage
-
Screening AthletesScreening AthletesScreening AthletesScreening
Athletes
Screening for SCDScreening for SCD8,000,0008,000,000 competitive
sports participants competitive sports participants
in the U.S. (high school & college)in the U.S. (high school
& college)
200,000200,000 estimated to be screened to estimated to be
screened to detect 1000 at risk and 1 who would diedetect 1000 at
risk and 1 who would diedetect 1000 at risk and 1 who would
diedetect 1000 at risk and 1 who would die
-
SCD in Young AthletesSCD in Young AthletesSCD in Young
AthletesSCD in Young Athletes•• Cost of NonCost of Non--Invasive
ScreeningInvasive Screening
–– 6,000,000 high school athletes6,000,000 high school
athletes••Screening exam $100Screening exam $100••ECG $70ECG
$70••Echocardiogram $1500Echocardiogram $1500••Total cost /
participant $1670Total cost / participant $1670
Total US health care cost Total US health care cost
$10,020,000,000$10,020,000,000
-
Screening athletesScreening athletesScreening athletesScreening
athletes•• Cost of NonCost of Non--Invasive ScreeningInvasive
Screeninggg
–– 10 million high school athletes10 million high school
athletes•• Screening exam $25Screening exam $25•• ECG $50ECG $50••
ECG $50ECG $50•• Echocardiogram $400Echocardiogram $400
Total US health care cost Total US health care cost $2 billion$2
billion to to prevent each preventable death.prevent each
preventable death.p pp p
(Per suspected athlete (Per suspected athlete
$330,000$330,000))
-
Normal Conduction Pathway Normal Conduction Pathway yyin the
Heart and the ECGin the Heart and the ECG
Sinoatrial (SA) NodeSinoatrial (SA) NodeAtrioventricular (AV)
Node
Left Bundle Branches
Right Bundle Branch
Purkinje Fibers
P = Atrial Depolarization
P T
QRS pQRS = Ventricular DepolarizationT = Ventricular
Repolarization
QRS
-
Normal Sinus RhythmNormal Sinus RhythmNormal Sinus RhythmNormal
Sinus Rhythm
Sinoatrial NodeSinoatrial NodeSinoatrial NodeSinoatrial Node
12:56 29MAR96 PADDLES X1.0 HR = 74
-
Ventricular TachycardiaVentricular TachycardiaVentricular
TachycardiaVentricular Tachycardia
12:57 29MAR96 PADDLES X1.0 HR = 214
-
Ventricular FibrillationVentricular FibrillationVentricular
FibrillationVentricular Fibrillation
12:57 29MAR96 PADDLES X1.0 HR = ---
-
Defibrillation: The OnlyDefibrillation: The OnlyDefibrillation:
The Only Defibrillation: The Only Effective Treatment for Effective
Treatment for
i l ib ill ii l ib ill iVentricular FibrillationVentricular
Fibrillation
300 JOULES DEFIB 20:29 01APR96 PADDLES X1.0 HR = ---
-
AsystoleAsystoleAsystoleAsystole
15:17 29MAR96 PADDLES X1.0 HR = ---
-
LIFEPAK® 500
Automated External DefibrillatorsDefibrillators
-
How to DefibrillateHow to DefibrillateHow to DefibrillateHow to
Defibrillate
•• Verify the victim is unconscious, not Verify the victim is
unconscious, not breathingbreathingbreathing, breathing, without a
pulse or signs of circulation without a pulse or signs of
circulation
•• Turn on AED and attach electrodesTurn on AED and attach
electrodes•• Turn on AED and attach electrodesTurn on AED and
attach electrodes•• ANALYZE heart rhythmANALYZE heart rhythm••
Follow the voice prompts and screenFollow the voice prompts and
screen•• Follow the voice prompts and screen Follow the voice
prompts and screen
messagesmessages
-
Defibrillation Electrode Defibrillation Electrode
PlacementPlacement
Anterior
Lateral
AnteriorAnterior--lateral placementlateral placement
-
Defibrillation Electrode Defibrillation Electrode
PlacementPlacement
Correct electrode position Incorrect electrode position
•• Correct electrode position optimizes the amount of Correct
electrode position optimizes the amount of current flowing through
the ventriclescurrent flowing through the ventricles
Correct electrode position Incorrect electrode position
-
Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac
DeathSudden Cardiac Death
•A public health crisis:
200000
250000
House fire
100000
150000Prostate cancer
Breast cancer
50000
100000Car accidents
Sudden cardiacarrest
0Annual incidence
arrest
-
S dd C di D thS dd C di D thSudden Cardiac DeathSudden Cardiac
Death
Why is early defibrillation so important?Chance of survival
Chance of survival from SCA from SCA diminishes 7diminishes
7--10%10%diminishes 7diminishes 7 10% 10% with every minute with
every minute after collapseafter collapse
-
Averting SCD in Young AthletesAverting SCD in Young
AthletesAverting SCD in Young AthletesAverting SCD in Young
Athletes
•• “Play Ball!” “Just DO it!”“Play Ball!” “Just DO it!”••
ScreenScreen appropriately with expert providersappropriately with
expert providersScreenScreen appropriately with expert
providersappropriately with expert providers•• Sometimes old
Sometimes old ––fashioned fashioned H&PH&P are the are
the
bestbestbestbest•• Keep Keep AEDAED handy, in working condition,
handy, in working condition,
and educate usersand educate usersand educate usersand educate
users
-
FINFINFINFIN
•• USA USA in Beijingin BeijingMarch 2008!March 2008!
-
ECG FindingsECG FindingsECG FindingsECG Findings
Distribution of 3 ECG categories with respect to sporting
disciplines among 1005 athletes. ECGs that were distinctly abnormal
(black bars), mildly abnormal (gray bars), and normal or with minor
alterations (white bars) are depicted as proportions of all the
athletes participating in each sporting discipline. Only sports
with 12 participants are shown. X-C indicates cross-country.