Transcript Adult with Congenital Heart Disease: Assessment of Function in

Pre-Sports Evaluation
Thomas R. Kimball, MD
Professor of Pediatrics
University of Cincinnati
Director
Echocardiography
Cardiovascular Imaging Core
Research Laboratory
Acknowledgement
• Waldemar Carlo
– Current PL-III at Cincinnati Children’s Hospital
– Future pediatric cardiology fellow at Texas
Children’s Hospital
Case Discussion
CC: Sports pre-participation physical
HPI: 15yo boy presents prior to football
season for you to fill out his preparticipation form. Has been healthy. Passed
out one time after feeling his heart racing
after running 5 miles last week.
ROS: any recent injuries, eye problems,
hypertension, chest pain
PMH: none
Case Discussion
Meds: none
FHx: no sudden deaths, no heart disease
SHx: denies steroids or supplements, drugs,
alcohol, smoking, sex
Physical Exam: normal
As the pediatrician, what do you do?
Outline
What are the
issues?
Problematic
Pediatric
Cardiovascular
Diseases
Current
Customary
Practice
•
•
•
Often, party interests are
competing (not
complementary)
Sports were not created all
equal
Leading causes of cardiac
sudden death:
– Hypertrophic
cardiomyopathy
– Coronary artery anomalies
•
Sports Intensity
Learning from
the Past: Profile
of Sudden
Death Victims
Future
Practice?
•
•
Sudden death victim profile:
– Asymptomatic high school
male
– Track, cross country,
basketball
Current practice:
– Hx, FH, PE
Future practice:
– Echo
Major Players
•
•
•
•
•
•
Athlete
Family
School/NCAA
Lawyers and Courts
Physicians
Consensus Guidelines
Unique Pressures for Primary Care
Physicians
• See many patients (high
denominator), low prevalence
of disease (small numerator)
• First symptom is frequently
sudden death
• Usually no physical findings
• Athlete may by stubborn
and/or non-compliant
Athlete’s Issues
Sahara Marathon
• Desire to play outweighs
almost every concern
• Spend enormous effort on
sport
• Self worth is wrapped up
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
• Event is riveting, puzzling and
challenging
• Intense interest may be
disproportionate to its actual
public health problem
Cost-Effectiveness Issues
• Not possible to achieve zero-risk
• Implied acceptance of risk on part of athlete
• Testing is expensive
– Occurrence of HCM is 1:500
– Echo ~$500
– $250,000 to detect even 1 previously undiagnosed case
• Problem of false positives
• F/U of abnormal results leads to more costly procedures
Scope of the Problem
• 200-300 young
athletes / year in USA
200,000 competitive
athletes screened
0.5%
1000 with CHD
1%
10 with disease capable of
causing sudden death
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 Intensity:
Static Classification
High
Static
Low
Static
Sports Intensity:
Dynamic Classification
Low
Dynamic
High
Dynamic
Sports Classification
MVC = maximum voluntary contraction
Max O2 = maximum oxygen consumption
Mitchell JH, et al. JACC 45:1364-67. 2005
Cardiac Etiologies of Sudden Death
in < 35 y.o.
ARVD
Poss myo
Myo
DCM
CAD
AS
Ao aneur
O ther
HCM
Cor Anom
Poss HCM
Marc-Vivien Foe
• Cameroon midfielder
• 28 y.o.
• Expires in 72nd minute in
soccer match vs.
Columbia in Lyon, France
in 6/2003
• 2 autopsies:
– No obvious cause of death
– Hypertrophic
cardiomyopathy
Hypertrophic Cardiomyopathy
• Relatively common 1:500
• Primary disease of cardiac
muscle (molecular defect
in cardiac sarcomere)
• LV hypertrophy without
dilatation
• More common in AfricanAmericans
• Male to female ratio of 9:1
for sudden cardiac death
• Autosomal dominant
Hypertrophic Cardiomyopathy
• Most common cause of SCD in athletes
• Patients with HCM who die suddenly:
– 70% die before 30 y.o.
– 50% show no limitations before death
– 40% engaged in physical activity
• Death probably due to dysrhythmia
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%
HCM
• History
– ½ pts are asymptomatic
– ½ pts have DOE, angina,
syncope, palpitations,
etc.
– FHx
• EKG
– LVH
• Signs
– Prominent LV impulse
– Frequently have no
murmur
– If present, murmur
increases with a decrease
in venous blood return
(supine  standing)
• ECHO
LVH and sudden cardiac death
From Spirito P, et al. NEJM 342:1778-1785, 2000.
Activity level and sudden cardiac
death in HCM
# of HCM patients
25
20
15
10
5
0
Sedentary
Walking
Adapted from Spirito P, et al. JACC 15:1521-6, 1990.
Exercise
HCM Treatment
• Treatment
– Medications (e.g.β-blockers) reduce symptoms but not
incidence of sudden death
– Ventricular septal myomectomy
– Alcohol septal ablation
• Avoid
– Competitive sports (except class 1A)
– Digitalis
– Diuresis/Dehydration
• Screen 1st degree relatives
Athlete’s Heart vs. HCM
Athlete’s Heart
HCM
LV thickness
< 16mm
> 16mm
LVH pattern
Concentric
Asymmetric
LV cavity
Large
Small
Diastolic Fxn
Normal
Impaired
Left Atrial Size
Normal
Dilated
Long QT Syndrome
• Ion channel mutation
• Delayed myocardial
repolarization
• Prolonged QTc
• Risk of Torsades
• QTc > 470 (men), 480
(women)
• Annual mortality rate
4.5%
Cardiac Events in Long QT
From Zareba W, et al. NEJM 339:960-965, 1998
SCD in Long QT syndrome
(particularly swimming)
Schwartz PJ, et al. Circulation. 2001;103:89.
Long QT Recommendations
• Symptomatic LQTS patients – Class 1A
• Asymptomatic LQTS patients with
prolonged QTc – Class 1A
• Genotype positive / phenotype negative
patients – no restrictions *
* Except no water sports for LQT1 patients
Implantable cardiac defibrillator
• Risk of ICD
damage/displacement
• Recommendations
– Class 1A sports only
Congenital Coronary Artery
Anomalies
Nl pattern
• Coronary arises from wrong
sinus
• Passes between great vessels
• Can be compressed when
cardiac output increased
• Can be surgically corrected
• EKG is usually normal
• Found in 1% of population
• Cause up to 20% of sudden
deaths on the athletic field
Single Coronary Artery
Pete Maravich – Atlanta Hawks, New
Orleans and Utah Jazz, Boston
Celtics, expired at 40 y.o. in 1988
during pick-up game
Anomalous Coronary Artery
• Possible Consequences
– Myocardial ischemia during exercise
– Ventricular tachyarrythmias from scarred
myocardium
• Recommendations
– No competitive sports
– Three months after surgical correction, may
participate in all sports, with normal maximal
stress testing
Kawasaki Disease
• Acquired coronary
artery aneurysm(s)
• Sports participation
depends on presence
and size of aneurysms
Hank Gathers
•
•
•
•
•
•
•
•
Basketball star for Loyola
Marymount University
In 1989, at 22 y.o. collapses during
LMU game against UCSB
Echo shows damaged area in LV
Diagnosed with exercise-induced
ventricular tachycardia, treated
with propranolol, LMU bought
defibrillator for courtside
Felt medication adversely affected
play, cut back on dosage
In 1990, at 23 y.o., collapses during
tournament game against Portland
DOA at hospital
Autopsy –
cardiomyopathy/myocarditis
Myocarditis
• Inflammatory disease of the myocardium
• Etiology
– Viral (enterovirus, parvovirus, adenovirus)
– Drugs
• Symptoms
– Chest pain, dyspnea on exertion, fatigue, syncope,
arrythmias, acute CHF
– Non-specific
Myocarditis
• Frequent cause of non-structural SCD
• Pathogenesis
– Myocardial inflammatory infiltrates, myocyte
necrosis, replacement fibrosis
– Arrythmogenic substrate
Recommendations
• 6 month off period
• Re-evaluation by cardiologist
–
–
–
–
EKG, ECHO
Stress test
Holter monitor
Serum markers of inflammation, heart failure
Flo Hyman
• American volleball player, 6’5”
• Known as “Clutchman” and
could spike ball at 110 mph
• Gold medal in 1982 World
Championship
• Silver medal in 1984 Olympic
Games
• Died at 31 y.o. after being
substituted for during a game in
Japan in 1986
• Aortic dissection due to Marfan
Syndrome
Marfan Syndrome
•
•
•
•
•
Connective tissue disorder
Autosomal dominant
Mutation in fibrillin-1 gene
Ocular, skeletal
Cardiovascular
–
–
–
–
–
Dilation of ascending aorta*
Aortic dissection*
Mitral regurgitation
Mitral valve prolapse
Abdominal aortic aneurysm
Recommendations
• Aortic root involvement
• Moderate/severe mitral
valve regurgitation
• FH of Marfan-related
sudden death or aortic
dissection
Class IA
Serginho
• Brazilian soccer player for
São Caetano
• Died on field at 30 y.o.
(2004)
• Autopsy reveals “enlarged
heart”
• Team owner and doctor
charged with homicide
ARVD
• 3rd leading cause of SCD in young athletes
• Prevalence
– 1 in 5000 in general population
• Pathology
– Fibrofatty replacement of RV myocardium
• Etiology
– Unclear
• Diagnostic Criteria
ARVD and exercise
• Fibrofatty RV is
arrhythmogenic
• Adrenergic stimulation
(exercise) induces
these arrhythmias
ARVD
• Prognosis
– 3% mortality rate
without treatment
– 1% mortality with
pharmacotherapy
• Treatment
– Beta Blockers
– Radiofrequency
ablation
– Implantable cardiac
defibrillator ?
– No athletic competition
except maybe class 1A
Sergei Grinkov
• Along with partner and
wife, Ekaterina Gordeeva,
three-time World Figure
Skating Pairs Champion
and 1988 and 1994 Winter
Olympic Champion
• Died suddenly at 28 y.o.
(1995) in Lake Placid
while practicing
• Autopsy – atherosclerotic
coronary artery disease
and hypertension
(diastolic of 110)
Commotio Cordis
Maron, B. J. et al. JAMA 2002;287:1142-1146.
Commotio Cordis
• Chest wall impact
• Rare but likely underreported
• Associated with competitive or recreational
athletics
Sports Participated in at the Time of
Commotio Cordis Events
Maron, B. J. et al. JAMA 2002;287:1142-1146.
Copyright restrictions may apply.
Age at Time of Commotio Cordis Event
Maron, B. J. et al. JAMA 2002;287:1142-1146.
Copyright restrictions may apply.
Pathophysiology
• No underlying heart
disease
• No major damage to
the heart or great
vessels
• Unimpressive force of
impact
Pathophysiology
• Transfer of energy
– Increased compliance of pediatric chest wall
• Energy of impact
– Greatest at around 30 - 50 mph
– Hardness
• Location – center of the heart
• Timing - repolarization
Prevention (?)
• Chest Wall Protectors
• Soft Balls
Cardiac
Etiologies
of Sudden
Death
Those
Etiologies
Readily
Detectable
in PE
< 35
y.o.
by Hx and
Screening
ARVD
Poss myo
Myo
DCM
CAD
AS
Ao aneur
O ther
HCM
Cor Anom
Poss HCM
Level of Competition
High
School
77%
College
3%
Pro
9%
Youth
11%
Sports in which Sudden Death Occurs
Other
Track
Football
Soccer
Baseball
Basketball
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 Preparticipation
Evaluation
• Identify individuals
– Known to be at risk
– Not known to be at risk
• Make recommendations
regarding participation
Legal Considerations
• Must use reasonable care
• No clear legal precedent
• Malpractice liability for failure to discover a
latent condition requires proof that a physician
deviated from customary medical practice
• Medical profession allowed to establish the
nature and scope of pre-participation screening
Risk Ratio between Athletes and
Non-Athletes
Athletes
Non-athletes
From Corrado D, et al. JACC 42:1959-1965, 2003.
What is “Customary Practice”?
Customary Practice
• No accepted standards
• Medical clearance by a
health care worker
consisting of H and P is
generally considered
customary
• In Ohio, the Ohio High
School Athletic
Association requires
completion of
preparticipation form
Limitations of Screening
•
•
•
•
False positives
Athlete disqualifications
Cost efficiency
Screening volume
American
Guidelines
(1996)
Family and personal history, physical
exam
Negative
Eligible for
competition
Positive
Further
testing
Negative
Positive
Further
management
European
Guidelines
(2005)
Family and personal history, physical
exam, and EKG
Negative
Eligible for
competition
Positive
Further
testing
Negative
Positive
Further
management
Efficacy of Screening with EKG
% of SCD attributed to HCM
30
25
20
Italy
USA
15
10
5
0
Athletes
Non-athletes
AHA Recommendations
• Preparticipation exam is
warranted
• Complete Hx, Family hx and
PE targeted to identify
cardiovascular lesions known
to cause sudden death
• (Noninvasive testing not
prudent in large populations)
• Repeat evaluation every 2
years
• Develop a national standard
for evaluation
Cardiovascular History
• Exertional chest pain,
syncope, or excessive
shortness of breath
• Detection of murmur or
hypertension
• FH of premature death or
disability < 50 y.o. or
specific knowledge of:
– HCM, DCM
– Long QT syndrome
– Marfan syndrome
Practical Tools
• Physical Activity
Readiness Questionnaire
(PAR-Q)
•
•
•
•
•
•
•
Has a doctor ever told you that you
have a heart condition and
recommended only medically
supervised activity?
Do you have chest pain brought on by
physical activity?
Have you developed chest pain in the
past month?
Have you on one or more occasions lost
consciousness or fallen over as a result
of dizziness?
Do you have a bone or joint problem
that could be aggravated by the
proposed activity?
Has a doctor ever recommended
medication for your blood pressure or a
heart condition?
Are you aware of any other physical
reason that would prohibit you from
exercising without medical
supervision?
• Stanford University
Pre-Participation
Form
• Internet-based
• Extensive (18 pages)
Cardiovascular Examination
•
•
•
•
BP
Auscultation
Femoral arteries
Marfan’s stigmata
Referral when
abnormalities in Hx
and PE
Noninvasive Screening Tests
• Echo will enhance
detection of abnormalities
–
–
–
–
Cardiomyopathy
AS
Aortic dilatation
Coronary artery anomalies
• But no guarantee
– Some coronary anomalies
– Arrhythmogenic RV
dysplasia
Echocardiogram
• Miniaturization of technology
• Targeted, limited examination
• Decreasing costs
Cost Effectiveness of
Screening Modalities
•
•
•
Med Sci Sports Exerc 32:887, 2000 Sensitivity (%)
(Sierra Heart Institute, Reno, NV)
High school athletes (HSA)
Specificity (%)
3 screening modalities
Screening cost ($)
– CV-specific Hx/PE
– EKG
– Echo
•
•
•
Abnl response cost ($)
Hx/PE
EKG
Echo
6
70
80
97.8
84.3
100
0
10
350
500
365
0
Assume 700,000 evaluations would
Years saved (yrs)
occur in search of 70 HSA
EKG is most cost-effective
To be equally cost-effective:
– Hx/PE would need 2X increase in
sensitivity
– Echo would need 4X decrease in
cost
10% - 40
90% - 20
Overall cost (mill $)
7.7
47.2
245
Years gained
93
1080
1232
84,000
44,000
200,000
Cost effectiveness
(cost/yr saved)
A Heart For Sports
•
•
•
Orange County, CA
Individual screenings (EKG and Echo) for $65 taxdeductible donation
“Recommended for”:
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If you want to learn more about your heart health
If you suffer high blood pressure, diabetes, sleep
apnea, high cholesterol, chronic lung condition,
alcohol dependency, smoke cigarettes, suffered a
previous stroke, or any heart condition
If you have a family history of heart disease, or sudden
death
If you have been diagnosed with a heart murmur
If you are an athlete and concerned about your heart
health
If you are not feeling well and are concerned about
your heart health
If you don’t know your Ejection Fraction (EF)
If you have not had an EKG or an echocardiogram in
the past year
If you are looking for peace of mind
Eddie Curry
• Chicago Bulls center
• 2 bouts of irregular heart
rhythm
• Suspected to have HCM,
genetic testing
recommended
• Curry refuses testing,
Bulls refuse to play him
• In 9/2005, traded to NY
Knicks who were willing
to play him (2005-2006
season: 72 games, 26
min/gm)
• “Genetic discrimination”
vs. privacy rights
Jason Collier
• #1 NBA draft pick of
Milwaukee Bucks in
2000
• Atlanta Hawks center
• Died on 10/15/05 (at
28 y.o.) at home
NBA Mandatory Screening
• Begins 2006 season
• Consists of:
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Personal and family hx
Physical examination
Blood work
EKG
Resting echo
Stress echo
• Administered annually
• If positive, no ban
• No training camp until
tests complete
Other League Policies
• MLB and NHL
– No uniform league-wide heart
screening program
• NFL
– Mandates cardiovascular exam and
EKG
– Partnered with Living Heart
Foundation
• Active and retired players
especially those at risk – i.e. large
body mass index
• Echo, EKG, Pulmonary Function
Test, Cholesterol Analysis,
Cardiac Risk Score, Blood
Glucose, Urine Drug check, Body
Fat and Body Mass Index, and
vital signs
NCAA
• Left to individual athletic
departments
• Georgia Tech
– Echo required for all
volleyball, basketball and
football
• Purdue
– 2.5 min echo ($35) on all
incoming athletes
• Ohio State University
– Currently performing echo
on every OSU athlete
(research study)
Will Kimble:
Athletics is His Life
•
•
•
•
•
•
In 2002 - starting center for Pepperdine
University
Fainted
Echo shows HCM, defibrillator placed,
restricted from playing
“I felt like I’d had something taken
away from me. It felt like the world
had just come down on me. I had
invested so much time and had worked
so hard”
Transferred to UTEP, NCAA grants
medical waiver, Kimble plays 2005
season
Not without controversy:
–
“The defibrillator was never designed to
operate in intercollegiate basketball.
The reliability is unknown” Barry
Maron, MD
Fred Hoiberg:
Risks Are Too Much
• Diagnosed with bicuspid aortic valve at
Iowa State in 1995
• Drafted by Pacers, traded to Bulls, then
Timberwolves
• Shooting guard, led league in 3-point
percentage in 2004-2005
• Echo as part of insurance policy in 2005
– Sinus of Valsalva aneurysm
• Surgery and pacemaker in 6/2005
• 1st NBA player to play with a
pacemaker???
• Announces retirement on 4/17/06
• Now coach for Timberwolves
Rony Turiaf:
Possible Success Story
• Signed rookie contract with LA
Lakers in 2005
• PE and echo show enlarged
aortic root
• Lakers void rookie contract
• However, Lakers also pay for
aortic root replacement
(7/26/05)
• In 1/06 signs new contract with
Lakers
• After rookie season played for
France in 2006 World
Championships
Take-Home Messages
• Sudden death is rare
• Issue of public perception (not
necessarily of public health)
• Most common causes are
– HCM
– Coronary anomalies
•
•
•
•
No legal precedent for malpractice
Standard care
Follow AHA recommendations
Refer to cardiology if any positive Hx,
FH, or PE
• Echo is becoming and will continue to
become more critical part of evaluation
Frequently Asked Questions
•
What are the American Heart Association
recommendations for preparticipation
evaluation?
–
•
What are the American College of Cardiology
recommendations for allowing participation in
the case of known cardiac disease?
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•
http://www.americanheart.org/presenter.jhtml?identifier=1478
Recommendations for Determining Eligibility for Competition in Athletes with
Cardiovascular Abnormalities: Bethesda Conference 26: (Revision of Bethesda
Conference #16), January 6-7, 1994. (J Am Coll Cardiol 1994;24:845-99)
What are the American College of Sports
Medicine recommendations for screening,
staffing and emergencies at health facilities?
– http://www.acsm-msse.org
•
Where can I find the Ohio High School Athletic
Association preparticipation form?
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•
http://www.ohsaa.org/medicine/physicalform.pdf
Where can I find the internet-based Stanford
University preparticipation form?
–
http://www.stanford.edu/dept/sportsmed/visitors/visitors98.html