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 DiscussionCC: Sports pre-participation physical

HPI: 15yo boy presents prior to football season for you to fill out his pre-participation 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 DiscussionMeds: 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?

OutlineWhat are the issues?

Sports Intensity

Problematic Pediatric Cardiovascular Diseases

Learning from the Past: Profile of Sudden Death Victims

• Often, party interests are competing (not complementary)

• Sports were not created all equal

• Leading causes of cardiac sudden death:

– Hypertrophic cardiomyopathy

– Coronary artery anomalies• Sudden death victim profile:

– Asymptomatic high school male

– Track, cross country, basketball

• Current practice: – Hx, FH, PE

• Future practice:– Echo

Current Customary Practice

Future Practice?

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

• Desire to play outweighs almost every concern

• Spend enormous effort on sport

• Self worth is wrapped up in sport

• Sense of invincibilitySahara Marathon

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

1000 with CHD

0.5%

10 with disease capable of causing sudden death

1%

1 with sudden death

10%

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

Mitchell JH, et al. JACC 45:1364-67. 2005

Sports Classification

MVC = maximum voluntary contraction

Max O2 = maximum oxygen consumption

HCM

Poss HCM

Cor Anom

O ther

Ao aneur

ASMyoPoss myo DCM

ARVD

CAD

Cardiac Etiologies of Sudden Death in < 35 y.o.

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 African-Americans

• 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

0

5

10

15

20

25

Sedentary Walking Exercise

# o

f H

CM

pati

ents

Adapted from Spirito P, et al. JACC 15:1521-6, 1990.

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

Schwartz PJ, et al. Circulation. 2001;103:89.

(particularly swimming)

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

• 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

Nl pattern

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)

Maron, B. J. et al. JAMA 2002;287:1142-1146.

Commotio Cordis

Commotio Cordis

• Chest wall impact

• Rare but likely underreported

• Associated with competitive or recreational athletics

Copyright restrictions may apply.

Maron, B. J. et al. JAMA 2002;287:1142-1146.

Sports Participated in at the Time of Commotio Cordis Events

Copyright restrictions may apply.

Maron, B. J. et al. JAMA 2002;287:1142-1146.

Age at Time of Commotio Cordis Event

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

Those Etiologies Readily Detectable by Hx and PE Screening

HCM

Poss HCM

Cor Anom

O ther

Ao aneur

ASMyoPoss myo DCM

ARVD

CAD

Cardiac Etiologies of Sudden Death in < 35 y.o.

Level of CompetitionHigh

School77%

Youth11%

Pro9%

College3%

Sports in which Sudden Death Occurs

BasketballFootball

Track

Other Soccer

Baseball

• Median age = 17 y.o.• Male (90%)• No obvious race

predilection• High school level of

competition• Asymptomatic (82%)• Sports

• Cross-country, track, basketball

Profile of the Athlete with Sudden Death

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

From Corrado D, et al. JACC 42:1959-1965, 2003.

Athletes

Non-athletes

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 PositiveEligible for competition

Further testing

Positive

Negative

Further management

European Guidelines

(2005)

Family and personal history, physical exam, and EKG

Negative PositiveEligible for competition

Further testing

Positive

Negative

Further management

Efficacy of Screening with EKG

0

5

10

15

20

25

30

Athletes Non-athletes

% o

f SCD

att

ribute

d t

o H

CM

I talyUSA

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 (Sierra Heart Institute, Reno, NV)

• High school athletes (HSA)• 3 screening modalities

– CV-specific Hx/PE– EKG– Echo

• Assume 700,000 evaluations would 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

Hx/PE EKG Echo

Sensitivity (%) 6 70 80

Specificity (%) 97.8 84.3 100

Screening cost ($) 0 10 350

Abnl response cost ($) 500 365 0

Years saved (yrs) 10% - 40 90% - 20

Overall cost (mill $) 7.7 47.2 245

Years gained 93 1080 1232

Cost effectiveness (cost/yr saved)

84,000 44,000 200,000

A Heart For Sports

• Orange County, CA• Individual screenings (EKG and Echo) for $65 tax-

deductible donation• “Recommended for”:

– 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:

– 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?

– http://www.americanheart.org/presenter.jhtml?identifier=1478

• What are the American College of Cardiology recommendations for allowing participation in the case of known cardiac disease?

– 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?– 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