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