Pre-Sports Cardiac Evaluation

Pre-Sports Cardiac Evaluation
Judith Lazol, MD
Pediatric Cardiology
Feb. 8, 20113
Outline
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What are the issues?
Sports intensity
Problematic Pediatric CV diseases
Learning from the past: profile of Sudden
Death Victims
 Current customary practice
 Future practice
Objectives
 Identify the most common cardiac causes
of sudden death in young athletes
 Define an appropriate cardiac evaluation
for participation in sports
 Distinguish the children that require
referral to a pediatric cardiologist
Pediatric Athletics:
What is Sudden Death?
 An unexpected and non-traumatic
event that occurs instantaneously or
within minutes of an abrupt change in
a person's previous clinical state.
Causes of sudden cardiac death in
young competitive athletes
 1435 athletes (1980-2005)
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Median age = 17 yrs
85% CARDIAC CAUSE
90% Male
44% African-American
68% Basketball or Football
90% died during training or
game
Maron, B. J. et al. Circulation 2007
Causes of sudden cardiac death in
young competitive athletes
HCM +
Coronary
anomaly
60%
< 35 years old
CAD
80%
> 35 years old
Maron, B. J. et al. Circulation 2007
Mar 27. 115 (12): 1643-1655
Incidence of SD in Athletes
Causes of sudden cardiac death in
young competitive athletes
Demographics
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Most athletes are of high school age at the
time of death
Majority of the athletes who incur sudden
death have been free of cardiovascular
symptoms
Maron, B. J. et al. Circulation 1996 94:850-856
Sports engaged in at the time
of sudden death
Maron BJ et al, JAMA 1996;276:199-204
Pediatric Athletics:
What’s the Sudden Death Risk?
 Athletes
 500,000 College and Pro Athletes
 5 million High school students play sports
 25 million children in competitive athletes
 ~ 30 student athletes die each year from
congenital heart malformations
ONE in a MILLION?!!!!
Cardiovascular Screening for High School Athletes
June 10 JAMA 1998;279:1817-1819
Causes of sudden cardiac death in
young competitive athletes
Here’s the Bad News
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158 athletes died
73% (115) had pre-participation physical
4 (3%) were suspected of CV disease
CV abnormality responsible for sudden
death was correctly identified in only 1
(0.9%)
Maron, BJ. et al. JAMA 1996;276;199-204
Pediatric Athletics:
What to Do?
 AMA (1998) assessment:
 20 states had no guidelines
 40% of High School associations did not properly screen
(≤ 4/12 of the AHA recommended elements)
 Update in 2005
 81% of states have adequate questionnaires (≥ 9/12)
 Circulation (2011)
 Survey of Pediatricians, family doctors & high school
athletic directors in Washington State
 <6% of PCP follow the national SCD screening guidelines
Major Players
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Athlete
Family
School/NCAA
Lawyers & Courts
Physicians
Consensus Guidelines
Unique Pressures for Primary Care
Physicians
 See many patients, low
prevalence of disease
 First symptom is
frequently sudden cardiac
death
 Usually no physical
findings
 Athlete may be stubborn
or non-compliant
Athlete’s Issues
Sahara Marathon
 Desire to play
outweighs almost
every concern
 Spend enormous
effort on sport
 Self worth is wrap in
sport
 Sense of invincibility
Problem of Public Health or Perception
 Athlete is a symbol
of health to society
 High visibility of
sudden death events
 High stakes of sports
as business
 Athlete has celebrity
status
Cost Effectiveness Issues
 Not possible to achieve zero-risk
 Implied acceptance of risk on part of the
athlete
 Testing is expensive
 Occurrence of HCM 1:500
 Echo ~$500
 $250,000 to detect 1 previously undiagnosed case
 Problem of false positives
 F/U of abnormal results leads to more costly
procedures
Scope of the Problem
200,000
Competitive athletes
screened
 150- 200 young athletes per year
1000
with CHD
0.5%
10 with disease capable
of causing SCD
1%
10%
1 with sudden
death
All Sports are not Created Equal
 Dynamic
 soccer, long distance running,
racquet sports
 Static
 weight-lifting, karate, water
skiing, gymnastics, field
events
 Combination
 football, sprint running
Sports Classification
MVC - Maximum voluntary Contraction
Max O2 – Maximum oxygen consumption
Mitchell JH, et al. JACC 45: 1364-67. 2005
Sudden Cardiac Death:
The Young and the Famous
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Hank Gathers (23, HCM, VT)
Reggie Lewis (27, Myocarditis)
Jim Fixx (52, Hypercholesterolemia, MI)
Sergei Grinkov (28, MI)
Krissy Taylor (17, ARVD)
River Phoenix (23, Heroin, Cocaine)
Jim Morrison (27, HF, drug overdose)
Jason Collier (28, Enlarged heart, arrhythmia)
Maggie Dixon, (28, Arrhythmia)
Andy Gibb (30, Myocarditis, cocaine abuse)
Pete Maravich (40, Coronary atresia)
Len Bias (22, Arrhythmia, cocaine)
Flo Hyman (31, Marfan, aortic dissection)
Sudden Cardiac Death:
The Young and the Famous
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Hank Gathers (23, HCM, VT)
Reggie Lewis (27, Myocarditis)
Jim Fixx (52, Hypercholesterolemia, MI)
Sergei Grinkov (28, MI)
Krissy Taylor (17, ARVD)
River Phoenix (23, Heroin, Cocaine)
Jim Morrison (27, HF, drug overdose)
Jason Collier (28, Enlarged heart, arrhythmia)
Maggie Dixon, (28, Arrhythmia)
Andy Gibb (30, Myocarditis, cocaine abuse)
Pete Maravich (40, Coronary atresia)
Len Bias (22, Arrhythmia, cocaine)
Flo Hyman (31, Marfan, aortic dissection)
Sudden Cardiac Death (SCD)
What Are We Screening For?
Structural/Functional
1) Hypertrophic Cardiomyopathy (HCM)
2) Coronary Artery Anomalies
3) Aortic Rupture/Marfan
4) Dilated Cardiomyopathy (DCM)
5) Myocarditis
6) Left Ventricular Outflow Tract
Obstruction
7) Mitral Valve Prolapse (MVP)
8) Coronary Artery Atherosclerotic
Disease
9)
Arrhythmogenic Right Ventricular
Cardiomyopathy (ARVC)
10) Post-operative Congenital Heart
Disease
Electrical
11) Long QT Syndrome (LQTS)
12) Wolff-Parkinson-White
Syndrome (WPW)
13) Brugada Syndrome
14)
15)
16)
Catecholaminergic Polymorphic
Ventricular Tachycardia (CPVT)
Short QT Syndrome
Complete Heart Block
Other
17) Drugs and Stimulants
18) Primary Pulmonary Hypertension
(PPH)
19) Commotio Cordis
Sudden Cardiac Death (SCD)
What Are We Screening For?
Structural/Functional
1) Hypertrophic Cardiomyopathy (HCM)
2) Coronary Artery Anomalies
3) Aortic Rupture/Marfan
4) Dilated Cardiomyopathy (DCM)
5) Myocarditis
6) Left Ventricular Outflow Tract
Obstruction
7) Mitral Valve Prolapse (MVP)
8) Coronary Artery Atherosclerotic
Disease
9)
Arrhythmogenic Right Ventricular
Cardiomyopathy (ARVC)
10) Post-operative Congenital Heart
Disease
Electrical
11) Long QT Syndrome (LQTS)
12) Wolff-Parkinson-White
Syndrome (WPW)
13) Brugada Syndrome
14)
15)
16)
Catecholaminergic Polymorphic
Ventricular Tachycardia (CPVT)
Short QT Syndrome
Complete Heart Block
Other
17) Drugs and Stimulants
18) Primary Pulmonary Hypertension
(PPH)
19) Commotio Cordis
Marc Vivien Foe
 Cameroon
midfielder
 28 y.o.
 Expires in 72nd
minute in soccer
match in Lyon,
France 6/2003
 Autopsy: HCM
Hypertrophic Cardiomyopathy
 Relatively common 1:500
 Primary disease of
cardiac muscle
(molecular defect in
cardiac sarcomere)
 LV hypertrophy without
dilatation
 More common in AA
 M:F 9:1 for sudden
cardiac death
 Autosomal dominant
Hypertrophic Cardiomyopathy
 Most common cause of SCD in young
athletes in the U.S.
 Patients who die suddenly
 70% die before 30 y/o
 50% show no limitations before death
 40% engaged in physical activity
 Death probably due to arrhythmia
HCM Phenotype
 Cardiac defects
 Abnormal cellular
architecture
 Hypertrophied LV
 Intramural
coronaries
 Risks
 Myocardial ischemia
 Arrhythmogenic
cardiac tissue
 LVOT obstruction
 Annual risk of SCD
is 1%
1 month
8 months
Hypertrophic Cardiomyopathy
 History
 ½ pts asymptomatic
 ½ pts DOE, angina,
syncope, palpitations, etc.
 FHx
 EKG
 LVH
 Abnormal in 75%-95%
(most are non-specific)
 Signs
 Prominent LV impulse
 Frequently have no
murmur
 If present, murmur
increases with decrease
in venous return (supine
to standing)
 Echo
LVH and sudden cardiac death
Spirito P, et al. NEJM 342: 1778-1785, 2000
Activity level and sudden cardiac death
in HCM
#
of
HCM
Pts
Spirito P, et al. JACC. 15:1521-26, 1990
Hypertrophic Cardiomyopathy
 Risk Factors for SCD (adult data)
 Prior arrest or spontaneous sustained VT
 Family history of premature HCM related
death
 Multiple syncopal episodes
 Multiple and repetitive non-sustained VT
 Hypotensive response to exercise
 Extreme LVH  30mm
 Early age at diagnosis
 Limited data in pediatrics
Hypertrophic Cardiomyopathy
 Treatment
 Medications (e.g. beta-blockers) reduce
symptoms but not incidence of sudden death
 Ventricular septectomy
 ICD
 Avoid
 Competitive sports (except 1A)
 Digitalis
 Diuresis/dehydration
 Screen 1st degree relatives
Long QT Syndrome
 Ion channel mutation
 Delayed myocardial
repolarization
 Prolonged QTc
 Risk of Torsades
 QTc > 470 men,
>480 women
 Annual mortality rate
4.5 %
Long QT Syndrome
 Incidence 1/3000
 Exercise/emotion/startle syncope or seizure
(occasionally misdiagnosed as neurologic or
vasovagal)
 Drowning/near drowning
 SIDS
 Congenital deafness
 Family history of SCD, seizure, syncope, LQTS
Congenital LQTS
Limitation of ECG Diagnosis
Johnson J N , Ackerman M J Br J Sports Med 2009;43:657-662
Cardiac Events in Long QT
Zareba, et al. NEJM. 339:960-965, 1998
Triggers of SCD in
Long QT Syndrome
particularly swimming
Schwartz, P. J. et al. Circulation 2001;103:89-95
Long QT Syndrome Therapy
 Symptomatic LQTS- Class 1A
 Asymptomatic LQTS w/ prolonged QTc- Class 1A
 Genotype positive/phenotype negative- no restrictions *
 Beta blocker
 Pacemaker, ICD or AED
 Avoidance of QT prolonging medications
* No water sports for LQT1 patients
Implantable Cardiac Defibrillator
 Risk of ICD
damage/displacement
 Recommendations
 Class 1A sports only
Congenital Coronary Abnormalities
 Coronary arises from
wrong sinus
 Passes between great
vessels
 Can be compressed when
CO increased
 Can be surgically
corrected
 EKG is usually normal
 Found in 1 % of
population
 Cause up to 20% of
deaths on the athletic field
Normal
LCA from
Rt sinus
RCA from
Lt sinus
Single Coronary Artery
 Pete Maravich 1947-1988
 Played for 3 NBA teams
 Died suddenly at age 40
during a pick up game
Congenital Coronary Artery
Anomalies
 Incidence 1/1,100
 Sudden death can be initial presentation
 Signs and Symptoms
 No Symptoms
 Syncope with exercise (most common)
 Chest pain with exercise (much less common)
 Variety of abnormalities can predispose to SCD
(especially if origin from the wrong sinus)
Congenital Coronary Artery Anomalies
 Usually have normal EKG, and stress test
 High index of suspicion
 Majority are amenable to surgical
correction
Anomalous Coronary Artery
 Possible consequences
 Myocardial ischemia during exercise
 Ventricular tachyarrhythmia from scarred
myocardium
 Recommendations
 No competitive sports
 May participate in all sports after surgery
with normal maximal stress testing
Kawasaki Disease
 Acquired coronary artery aneurysm
 Sports participation depends on presence
and size of aneurysm
Coronary Complications
Coronary
stenosis
Saccular
Aneurysms
 Reggie Lewis
1965-1993
 Died @ 27
during an off
season practice
 Collapse during
a play-off in the
preceding mos
 Cardiomyopathy
2* adenovirus
Myocarditis
Myocarditis
Myocarditis
 Inflammatory disease of the myocardium
 Etiology
 Viral (enterovirus, parvovirus, adenovirus)
 Drugs
 Symptoms
 Chest pain, DOE, fatigue, syncope,
arrhythmias, CHF
 Non-specific
Myocarditis
 Frequent cause of non-structural SCD
 Pathogenesis
 Myocardial inflammatory infiltrates,
myocyte necrosis, replacement fibrosis
 Arrhythmogenic substrate
Recommendations
 6 month off period
 Re-evaluation by
cardiologist
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EKG, Echo
Stress test
Holter monitor
Serum markers of
inflammation, heart
failure
Flo Hyman
 American volleyball
player, 6’5’’
 Gold medal in 1982
World Championship
 Silver medal in 1984
Olympic games
 Collapsed while sitting
on a sideline
 Died at the age of 31
 Aortic dissection due to
Marfan
Marfan Syndrome
 CT disease
 1 in 5,000 to 10,000
 AD w/ highly variable
clinical presentation
 1/3 of cases represent
new mutations
 CV
 Dilatation of Ascending
aorta/Aortic dissection
 MVP, MR
 Dilatation of MPA
Recommendations
 Aortic root
involvement
 Moderate/severe
MV regurgitation
 FH of Marfan
related death or
aortic dissection
ARRYTHMOGENIC RIGHT
VENTRICULAR DYSPLASIA
 3rd leading cause of SCD in young athletes
 Prevalence
 1 in 5000 in general population
 Pathology
 Fatty-fibrous replacement of myocytes in the right
ventricular free wall
 Etiology- unclear
 Familial occurrence
 Recurrent or intractable ventricular arrhythmias
ARRYTHMOGENIC RIGHT
VENTRICULAR DYSPLASIA
Diagnosis
 ECG: T- wave
inversions in V1 and
V2 and isolated PVC s
are common as well
as LBBB morphology
 MRI: more reliable
than echo in
noninvasive diagnosis
ARRYTHMOGENIC RIGHT
VENTRICULAR DYSPLASIA
 Prognosis
 3% mortality rate
without treatment
 1% mortality rate with
pharmacotherapy
 Treatment
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Beta blockers
RFA
ICD
No athletic competition
except maybe class
1A
Commotio Cordis
Maron: N Engl J Med, Volume 349(11).September 11, 2003.1064-1075
Commotio Cordis
 “Blunt, non-penetrating, and innocent blows to
the chest that produce ventricular fibrillation,
unassociated with structural injury to the ribs,
sternum, or heart.”
 Most common in children and adolescents
 Survival rare (15%) of reported cases
Maron: JAMA, Volume 287(9).March 6, 2002.1142-1146
Pathophysiology
 No underlying heart
disease
 No major damage to
the heart or great
vessels
 Unimpressive force of
impact
Age & Type of Activity
224 cases National Commotio Registry
(15 years)
Maron: NEJM, Volume 362(10).March 11, 2010. 917-927
Sports Participated in at the Time of
Commotio Cordis Events
Maron: NEJM, Volume 362(10).March 11, 2010. 917-927
Commotio Cordis
 AED’s can reduce
mortality
 Prevention
 education
 soft balls
yes
 chest protectors
inadequate
Maron: NEJM, Volume 362(10).
March 11, 2010. 917-927
Profile of the Athlete with Sudden
Death
 Median age= 17 y/o
 Male (90%)
 No obvious race
predilection
 High school level of
competition
 Asymptomatic (82%)
 Sports
 Cross-country, track,
basketball
Purpose of Pre-participation Evaluation
 Identify individuals
 known to have risk factors
 Not known to be at risk
 Make recommendations regarding
participation
Can We Really “Clear”
Someone to Play Sports?
“Medical Clearance is not a promise that the
athlete is free from potentially fatal
cardiovascular disease. Thus, we need to
acknowledge that, albeit potentially reduced,
small risks remain for potentially fatal disease,
even after a normal screening examination”
Maron, B.J. Cardiovascular risks to young athletes on the
athletic field. Ann Intern Med 1998;129:379-86
Legal Considerations
 Must use reasonable care
 No clear legal precedent
 Malpractice liability for failure to discover a latent
condition requires proof that the physician
deviated from customary medical practice
 Medical profession allowed to establish the
nature and scope of pre-participation screening
Pediatric Athletic Screening: AHA 12 element
preparticipation cardiovascular screening
 History (parental verification)
 Personal
1. Exertional chest pain/discomfort
2. Unexplained syncope/near syncope (not
neurocardiogenic/vasovagal)
3. Excessive exercise dyspnea or fatigue
4. Prior recognition of a heart murmur
5. Hypertension
Circulation 2007;115:000-000
Pediatric Athletic Screening: AHA 12 element
preparticipation cardiovascular screening
 History (parental verification)
 Family
6. Premature death (sudden, unexpected or
otherwise) < 50 y/o in ≥ 1 degree relative
7. Disability from heart disease <50 in close
relative
8. Specific knowledge of an inherited cardiac
condition (HCM, DCM, LQTS, channelopathies,
Marfan Syndrome, clinically impt arrhythmias)
Pediatric Athletic Screening: AHA 12 element
preparticipation cardiovascular screening
 Physical Examination
9.
10.
11.
12.
Heart murmur (supine and standing or w/ valsalva)
Femoral pulses to exclude aortic coarctation
Evaluate for physical stigmata of Marfan Syndrome
Brachial BP in seated position (preferably both arms)
Circulation 2007;115:1643-1655
AHA Cardiovascular PPE
Recommendations
 1st yr at institution/high school:
 Comprehensive personal and family history
 Physical examination by qualified examiner
 CV PPE every 2 years after initial screening
 During intervening years: history
 Refer when abnormal
Pediatric Athletic Screening:
Common Red Flags
 Exertional
 Syncope
 Chest Pain
 Dyspnea
 Palpitations
Pediatric Athletic Screening:
Common Red Flags
Exertional Symptoms
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ECG, Echo, Stress Test
± TEE, coronary angiogram
± Nuclear test
± Electrophysiology study
Non invasive Screening Test
 Echo will enhance
detection of
abnormalities
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CM
AS
Aortic dilatation
Coronary artery
abnormalities
 But no guarantee
 Some coronary anomalies
 ARVD
Limitations in Screening
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False negatives
False positives
Athlete disqualification
Cost efficiency
Screening volume
The current US approach to PPE
The Italian approach to PPE
Cost Effectiveness of Screening
Modalities
EKG is most
cost-effective
To be equally
cost- Effective:
 Hx/PE need
2x inc in
sensitivity
 Echo needs
4x decrease
in cost
FULLER: Med Sci Sports Exerc, Volume 32(5).May 2000.887-890
Barriers to routine ECG-based
screening
 Large number of athletes for the size of
appropriate physician work force
 Lack of standardization for interpretation
of ECGs in athletes
 Lack of normative data in certain
demographic and ethnic group
Mandatory ECG Screening
(?) reduce the risk of SCD
 Israel Sport Authority
 Results compared 12 yrs
before & after 1997
legislation
 Mandatory screening with
resting ECG & exercise
testing
 Mandatory ECG screening
of athletes had
no apparent effect
on the risk of cardiac death
1985-2009
Before 1997
After 1997
11
13
2.54 events
2.66 events
p=0.88
Steinvil, et.al. JACC. 2011; 57: 1291-1296
Annual Incidence of Sudden Cardiac Death Expressed per 100,000 PersonYears in the 3 Studies Evaluating the Effects of Screening on the Mortality of
Athletes Over Time
Steinvil, et.al. JACC. 2011; 57: 1291-1296
NBA Mandatory Screening
 2006 Season
 Consists of
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Personal & Family Hx
PE
Blood work
EKG
Resting echo
Stress echo
 Administered annually
 No training camp until
complete
Other League Policies
 MLB and NHL
 No uniform league wide
screening program
 NFL
 Mandates CV exam &
EKG
 Partnered w/ Living
Heart Foundation
 Active & retired players
esp those at risk- e.g.
large BMI
 NCAA
 Left to individual athletic
departments
Take Home Message
 Sudden Death is rare
 Issue of public perception (not
necessarily public health)
 Most common cause
 HCM
 Coronary abnormalities
 No legal precedent of
malpractice
 Follow AHA recommendations
 Refer to Cardiology for any
(+) Hx, FHx, or PE
THANK YOU