Sudden Death in the Young Athlete

9/17/2010
Phidippides
Phidippides
Sudden Death in the Young Athlete
What is the extent of the problem?
Can we prevent it?
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Number of cardiovascular (CV), trauma-related, and other sudden death events
in 1866 young competitive athletes, tabulated by year (1980-2006)
Maron, B. J. et al. Circulation 2009;119:1085-1092
Copyright ©2009 American Heart Association
Approximately 1/200,000 in USA
Reported rate increasing 6%/year
• 65% < 18 years old, 59% high school sports
• 89% male
• Basketball(33%) and football(25%) most common (soccer outside U.S.A.)
(
id
S )
• 80% during or just after sports or physical activities, 20% not associated with activity
• Incidence 0.61/100,000 person years (2.3/100,000 person years in Italian lit.)
Incidence and relative risk (RR) of sudden death (SD) among athletes (solid columns) and
non-athletes (open columns) from cardiovascular and non-cardiovascular causes
Does exercise
increase the risk
of sudden death
in athletes?
Corrado, D. et al. J Am Coll Cardiol 2003;42:1959-1963
Copyright ©2003 American College of Cardiology Foundation. Restrictions may apply.
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Causes of Sudden Death
in 387 Young Athletes.
Maron BJ. N Engl J Med
2003;349:1064-1075.
Distribution of cardiovascular causes of sudden death in 1435 young
competitive athletes
Maron, B. J. et al. Circulation 2007;115:1643-1655
Copyright ©2007 American Heart Association
National Registry of
Sudden Death in the Athlete
• The athlete participated in organized team or individual sports that required regular competition against others as a central component, placed a high premium on excellence and achievement and
premium on excellence and achievement, and required vigorous, systematic training.
• Sudden death or survived cardiac arrest at < 40 years old. Sudden death was defined as an unexpected collapse with or without physical exertion with no previous history
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Flow diagram summarizing causes of death in 1866 young competitive athletes
Maron, B. J. et al. Circulation 2009;119:1085-1092
Copyright ©2009 American Heart Association
Ray Chapman –Shortstop, Cleveland Indians
August 17th 1920
Cardiovascular deaths according to race, with respect to the number of white
and nonwhite athletes with each disease
Deaths due to CV disease
64% of non-whites
51% of whites
Maron, B. J. et al. Circulation 2009;119:1085-1092
Copyright ©2009 American Heart Association
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Classification of sports Recommendations
1A
Mitchell, J. H. et al. J Am Coll Cardiol 2005;45:1364-1367
Copyright ©2005 American College of Cardiology Foundation. Restrictions may apply.
Hank Gathers
LMU
1967-1990
NCAA Leader in scoring
and rebounding
Hypertrophic
Cardiomyopathy
Inappropriate LV Hypertrophy (without cause) without
dilatation
With hyperdynamic systolic function
1/500 of US population
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Hypertrophic Cardiomyopathy
Synonyms
• Idiopathic Hypertrophic Idiopathic Hypertrophic Subaortic
Subaortic Stenosis (IHSS)
• Hypertrophic Obstructive Hypertrophic Obstructive Cardiomyopathy
Cardiomyopathy (HOCM)
• Asymmetric Hypertrophic Asymmetric Hypertrophic Cardiomyopathy
Cardiomyopathy (ASH)
Genetic Abnormality in HCM
• Inherited trait is usually autosomal dominant.
• HCM is frequently a hereditary disorder with
transmission to 1st degree relatives in 50% of
cases. Geneticallyy and p
phenotypically
yp
y HCM is
extremely heterogeneous disease.
• Mutations in genes for cardiac sarcomeric
proteins are linked to HCM. (12 identified)
Largest number of mutations have been
identified in ß-myosin heavy chain gene on
chromosome 14.
Diagnosis of HCM
• Variants of HCM – Asymmetric
hypertrophy of LV, predominantly
septum and anterior wall (70%); basal
septal hypertrophy (15
(15-20%);
20%);
concentric left ventricular hypertrophy
(8-10%); apical and lateral wall
(<2%).
• Extent of hypertrophy inversely
related to age*
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Hypertrophic Cardiomyopathy
Obstructive (25%) vs Non-Obstructive
Hypertrophic Cardiomyopathy
Hypertrophic
Cardiomyopathy
Increased Myocardial O2
Demand
Decreased diastolic function
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D
S
Hypertrophic Obstructive Cardiomopathy
Dynamic Outflow Obstruction
Hypertrophic
Obstructive
Cardiomyopathy
Hypertrophic Cardiomyopathy
Degree of Obstruction
•
Directly related to degree of contractility
•
Inversely related to degree of vascular
resistance ((afterload
afterload))
•
Inversely related to degree of left
ventricular filling (preload)
Consider relationship to the physical activity
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Pathophysiology of HCM
• Dynamic left ventricular outflow tract
(LVOT) obstruction (25%).
• Diastolic dysfunction
(Impaired Relaxation and Compliance)
• Arrhythmias – PVC’s, VT, Atrial fibrillation
• Myocardial ischemia (usually with normal
coronary arteries).
• Mitral regurgitation (MR).
Symptoms of HCM
1. Fatigueability
2. Angina Pectoris
3. Dyspnea
y p
4. Palpitations
5. Syncope, sudden death
Most patients are asymptomatic
Incidental finding on exam, EKG, Echo
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Hypertrophic Cardiomyopathy
EKG
Left ventricular hypertrophy
Q Waves
ST Segment and T Wave Abnormalities
Arrhythmias
Left bundle branch block
EKG is frequently normal or non-diagnostic
Systolic anterior motion
SAM
Prognosis of
HOCM in Unselected Populations
Annual Cardiovascular Mortality
2.0
1.5
%/yr 1.0
0.5
0.0
Study Doffland
Pt (no.)
113
Spirito
151
Cecchi
202
Cannan
38
Maron
234
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Survival in a Regional U.S. Cohort
Age at Diagnosis
>20 Years
N = 234
Age at Diagnosis
<20 Years
N = 43
Maron: JAMA, 1999
Survival According to Clinical Variables
Maron et al. JAMA 1999;287:650
1999;287:650--655
Highest Levels of Risk in HOCM
Prophylactic use of AICD
•
Previous cardiac arrest
•
Family history of premature HCMHCM-related death in (or
multiple) relatives
•
DNA documentation of certain highhigh-risk mutant genes
•
Syncope
•
Multiple bursts of NonNon-sustained VT
•
LVH >30
>30 mm wall thickness
•
Hypotensive BP response to exercise
•
EPS response is an unresolved issue “and has been largely
abandoned”.
after Maron
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Recommendations for HCM
Class IA

Competitive sports are contracontra-indicated
regardless of age, gender, phenotypic
appearance, symptoms, LVOT
obstruction, or prior treatment with
medical therapy or interventions such as
AICD, surgical myectomy or ETOH septal
ablation.
Myocarditis
•
•
•
Inflammatory disease of the myocardium,
usually due to a viral infection
Evolves through
g an acute inflammatory
y
process followed by heterogeneous
necrosis and healing with interstitial
edema, focal necrosis, and replacement
fibrosis. (unstable electrical substrate)
May progress to dilated cardiomyopathy
Myocarditis
•
•
•
•
•
Antecedent viral illness
Chest pain, DOE, fatigability, palpitations,
syncope
EKG: conduction abnormalities,,
supraventricular and ventricular
arrhythmias
ECHO: Global or regional abnormalities,
dilatation, pericardial effusion
CHF, Cardiogenic shock
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Myocarditis
Diagnosis (Extremely difficult)
•
•
•
•
Clinical evaluation
EKG,
EKG echocardiogram
Myocardial biopsy
Cardiac MRI
Myocarditis
Recommendations
•
•
•
•
Competitive sports are contraindicated
for 6 months after onset of symptons
Resolution of EKG and ECHO
abnormalities at rest (including
arrhythmias)
No inducible EKG or ECHO abnormalities
with exercise testing
Resolution of inflammatory markers
Pericarditis
Recommendations
Competitive sports contracontra-indicated until
complete resolution of clinical course as
well as EKG, ECHO and biomarker
abnormalities
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Flo Hyman
1954‐‐1986
1954
Olympic Volleyball
Olympic Volleyball
1980 and 1980 and 1984
1984
Marfan Syndrome
•
•
•
•
Hereditary autosomal disorder (<1:5000)
>400 Mutations
Progressive aortic root dilatation of
ascending or descending aorta
predisposing to dissection or rupture
Mitral valve prolapse with associated
significant regurgitation and/or left
ventricular dysfunction may be present
Marfan Syndrome
Diagnosis
•
•
•
•
•
History (Family history)
Physical – Aortic or mitral regurgitation,
other unique Marfan features
Chest XX-Ray
Echocardiogram
CT Angiography / MRA
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Marfan Syndrome
Recommendations
•
•
•
•
Competitive sports contracontra-indicated if:
a) aortic root dilatation
b) moderate to severe regurgitation
c) family history of dissection
d) family history of sudden death ((Marfan
Marfan))
Low to moderate sports only without
above
Avoid sports involving body collision
Regardless of medical or prior surgical
therapy
“Pistol Pete” NBA Guard
Pete Maravich 1947-88
Anomalous Coronary Artery
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Single Coronary Artery
•May have exertional symptoms
•May have abnormal exercise test
A 15-year-old male Italian
soccer player with a
history of exertional
syncope one year before
death who died suddenly
while running during the
second half of a game
Basso, C. et al. J Am Coll Cardiol 2000;35:1493-1501
Copyright ©2000 American College of Cardiology Foundation. Restrictions may apply.
Congenital Coronary Anomalies
•
•
•
Coronary arteries arising from the wrong
coronary sinus
Competitive sports is contra
contra--indicated if
the coronary
y artery
y traverses between the
Aorta and Pulmonary Artery
Can resume sports 3 months after
successful intervention if no evidence of
residual inducible ischemia, arrhythmias
or myocardial damage
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Arrhythmogenic Right
Ventricular Dysplasia (ARVD)







Replacement of RV myocardium with fatty and
fibrotic tissue and thinning causing electrical
instablility
Autosomal dominant disorder
Common in Italian literature (6/10,000)
Difficult to diagnose clinically
Difficult to diagnose nonnon-invasively
Syncope/ family history important
Competitive sports contracontra-indicated
Congenital Heart Disease
•
•
•
•
•
•
Atrial septal defect
Ventricular septal defect
Aortic valve disease
Mitral valve disease
Coarctation of aorta
Patent ducus arteriosus
Congenital Heart Disease-untreated
Recommendations
Competitive sports are contracontra-indicated if
the lesion is hemodynamically significant
 Extensive nonnon-invasive evaluation is
critical and frequently involves exercise
testing as well
 Physical examination with careful cardiac
auscultation is critical in the screening
process

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Primary cardiac arrhythmias
Clinical Evaluation
•
•
•
•
Frequently asymptomatic
Symptoms such as palpitations are
frequently
q
y benign
g
Syncope requires extensive evaluation
Family history of sudden death requires
further evaluation
Primary cardiac arrhythmias
•
•
•
•
•
•
•
Sinus nodal dysfunction
AV nodal dysfunction
Atrial arrhythmias
Ventricular arrhythmias
Accessory bypass tracts (WPW)
Ion channel disorders
Brugada
Sinus and AV Nodal Dysfunction in
the Trained Athlete
•
•
•
•
Very common due to high vagal tone
Resolution should occur with exercise testing.
If not, further evaluation may be needed to clear
for sports
Symptoms of ALOC, fatigue, etc., should
warrant further evaluation
Congenital, narrow QRS Complete AV Nodal Block with
no symptoms, structurally normal heart, and
appropriate response to exercise testing are cleared for
sports
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Athletes with Pacemakers
•
•
Sports with high likelihood of bodily
collision with potential direct blow to
generator are contracontra-indicated ( Football,
rugby, boxing, lacrosse, martial arts and
h k )
hockey)
Protective padding for the generator is
recommended for other sports with
possible bodily collision ( Baseball,
softball, soccer, and basketball)
WPW (Pre-excitation) Syndrome
Accessory Bypass Tract = Beta pathway
AV Node = Alpha Pathway
Early, but
Abnormal
ventricular
depolarization
Accessory bypass tracts
Prone to atrial
fibrillation
Can result in very
rapid ventricular
response and
sudden death
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Accessory Bypass Tracts (WPW)
Symptoms or EKG needed to identify in advance
•
•
•
•
If > 20 years old, asymptomatic, no evidence of
structural heart disease and no inducible
arrhythmias - can participate in competitive
sports.
Further evaluation, possible invasive, in those
< 20 years old
Symptoms or inducible arrhythmias requires
further evaluation
Successful ablation allows full participation in
sports without restriction
Ventricular Ectopy
•
•
PVC’s as well as ventricular
tachyarrhythmias are common in the
trained athlete. Usually no associated
structural heart disease and do not
appear to be at increased risk. Frequently
disappear with deconditioning.
deconditioning.
H&P, EKG, ECHO and exercise testing
are usually needed for evaluation
Hereditary Arrhythmias
•
•
•
Long QT Syndrome ((QTc
QTc > 470ms)
Brugada Syndrome
Competitive sports usually contracontraindicated
QTc = QT/√R
QT/√R--R Interval
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Demographics and Natural History
of Congenital LQTS
•
1:10,000 is gene carrier
•
Estimated incidence of sudden death
3-4,000/year
•
Untreated patients incidence of sudden
cardiac death is 10%/year
•
30% of deaths or aborted deaths occur as
first event
LQTS: Phenotype-Genotype
Considerations
•
•
•
•
•
6 genotypes; ~200 different mutations
Clinical differences among LQT1, LQT2, & LQT3
genotypes
g
yp
Clinical variability within a genotype
Clinical variability among members of a family
with the same gene mutation suggests presence
of modifier genes
If asymptomatic, restricted to 1A activities
Occurrence of
Gene-Specific Triggers
70
Exercise
Emotional Stress
Rest
60
Perce
ent
50
40
30
20
10
0
LQT1
LQT2
LQT3
Circ 2001;103:892001;103:89-95
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Chromosome 3
II
aVF
V5
Event--Free Survival in 580 Patients According to
Event
the Genetic Locus
No. at Risk
LQT1
LQT2
LQT3
355
176
49
249
130
30
192
187
20
146
57
9
100
34
7
Priori et al., NEJM 2003;348:18662003;348:1866-72
Event--Free Survival Among 580 Patients
Event
According to Quartile QTc
446 msec or less
447--468 msec
447
468--498 msec
468
>498 msec
No. at Risk
1st quartile
2nd quartile
3rd quartile
4th quartile
148
150
140
142
112
104
103
92
96
80
78
45
76
62
49
28
45
45
33
18
Priori et al., NEJM 2003;348:18662003;348:1866-72
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Brugada Syndrome
The syndrome is a clinical
clinical-electrocardiographic diagnosis based on
the occurrence of syncopal or sudden
death episodes in patients without
demonstrable structural heart disease and
a characteristic ECG pattern of apparent
right bundle branch block and ST segment
elevation in leads V1 to V3.
Two Types of ST Segment Elevation From Patients
with Brugada-Type LCG in leads V1 to V3
Coved
Type
SaddleSaddleCoved
back
Type +
Type Pilsicainide
V1
V2
V3
Ikeda ANE 2002;7:2512002;7:251-262
Risk Stratification in Brugada
•
•
•
•
•
•
•
Common abnormality ? (Italian lit.)
SCD tends to occur around age 40
Males > Females (9:1)
FH common but not predictive of fatal events
Highest risk in pts with resuscitated SCD or
syncope with spontaneous pattern
No effective drug therapy – only ICD
Restricted to 1A sports activities only
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Preparticipation Screening
•
•
•
History and Physical Examination
Formalized routine screening including
EKG’s and Echocardiograms?
DNA Testing?
*10--12 million athletes eligible for evaluation
*10
*0.3% incidence of cardiac diseases capable
of causing sudden death
Only 3% of screened trained athletes who died suddenly
of heart disease were suspected of possible CV disease
and none were disqualified from competition
History Screening
•
•
•
•
•
•
Symptoms: exertional chest pain, excessive
exertional dyspnea
dyspnea,, inappropriate fatigue,
palpitations, altered loss of consciousness,
syncope
History of heart murmur
Family history: Premature heart disease and/or
unexpected sudden death < 50 years old
Family history of cardiomyopathy,
cardiomyopathy, ion
channelopathy,, Marfan Syndrome
channelopathy
Drug history (Cocaine abuse, etc.,)
Co--morbid conditions
Co
AHA Consensus Panel Recommendations 2005 Bethesda Conference
Physical Examination
•
•
•
•
Elevated blood pressure, heart rate
Peripheral pulses ((Coarctation
Coarctation of aorta)
Stigmata of Marfan syndrome
Cardiac auscultation
a) Significant irregularities
b) Heart murmur: DYNAMIC auscultation
JACC Vol.45. No.8,2006
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Pre-participation Screening
High School/ College
•
•
•
•
41 states have adequate questionnaires
(> 9 of 12 questions as recommended by AHA)
4 states inadequate with < 4 questions
No national mandate, governed state by state
with
ith 64% iincrease iin nonnon-physicians
h i i
performing evaluation in past decade, 18 states
allow screening by chiropractors and
naturopaths
NCAA schools now routinely use mandated
screening measures including 10 of 12
questions (excluding Marfan and fatigueability)
fatigueability)
Gaines Adams 19831983-2010
Defensive End Chicago Bears
Thomas Herrion 1981
1981--2005
Offensive guard SF 49’s
Athlete’s Heart
Physiological adaptations and cardiac structural
remodeling in trained athletes can result in
cardiac arrhythmias and morphological
abnormalities that can closely resemble
serious cardiovascular diseases such as
hypertrophic, dilated, and arrhythmogenic right
ventricular cardiomyopathies as well as sinus
and complex ventricular dysrhythmias that may
result in inappropriate disqualification of
competitive sports.
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Gray Area of Overlap between Athlete's Heart and Cardiomyopathies,
Including Myocarditis, Hypertrophic Cardiomyopathy, and Arrhythmogenic
Right Ventricular Cardiomyopathy.
Maron BJ. N Engl J Med 2003;349:1064-1075.
Sudden death in the competitive athlete is
fortunately a rare event. The death of an
athlete stirs public interest and arouses
emotions about a young life, especially
someone who should be in better health
than the average person, needlessly lost.
The approach to prevent these events is
controversial.
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