THE SCOPE OF THE PROBLEM Athlete Deaths Phidippides 530 BC 490 BC - PowerPoint PPT Presentation

Headlines you DON’T want to see Theodore P. Abraham, MD, FACC Meyer Friedman Distinguished Professor of Medicine UCSF HCM Center of Excellence University of California at San Francisco 6/18/2018 Disclosures: none THE SCOPE OF THE PROBLEM Athlete Deaths – Phidippides 530 BC – 490 BC  3000 sudden deaths per year in ages 15-34 years (CDC)  125-300 high school athletes a year (informal estimates)  The population  4 million competitive high school–age athletes (grades 9 - 12)  ~500 000 collegiate  ~ 5000 professional  unknown # of youth, middle school, and masters level competitors  Prevalence of field deaths uncertain  estimated 1:100 000 to 1:300 000 high school–age athletes  disproportionately higher in males  Older athletes estimated at  1:15, 000 in joggers  1:50, 000 in marathon runners Maron 1980, 1996 June 18, Thompson 1982 3 2018 Van Camp 1995 1

WHY EVALUATE? THE DISEASE MIX  Purpose of screening  provide medical clearance in competitive sports  < 35 years - identify clinically relevant and preexisting cardiovascular abnormalities  Hypertrophic cardiomyopathy ~ ⅓ - reduce the risks associated with organized sports.  Rationale  Coronary artery anomalies  > 35 years  intense athletic training >> increases risk for sudden cardiac death/disease progression  Atherosclerotic heart disease  not possible to quantify that risk  Deaths most common in basketball  majority of young athletes die during athletic training or competition and football  early detection permits timely interventions that may prolong life. Maron 1996 Maron 1980 Burke 1991 Van Camp 1995 Medical history (with parental confirmation) AHA VIEWS ON SCREENING 12-element Personal history 1. Exertional chest pain/discomfort AHA Some form of pre-participation cardiovascular 2. Unexplained syncope/near-syncope (not neurocardiogenic/vasovagal) screening for high school and collegiate athletes is screening 3. Excessive exertional and unexplained dyspnea/fatigue, associated with exercise justifiable and compelling, based on ethical, legal, guide 4. Prior recognition of a heart murmur and medical grounds. 5. Elevated systemic blood pressure Family history a) Noninvasive testing can enhance the diagnostic power 6. Premature death (sudden and unexpected, or otherwise) before age 50 years due to of the standard history and physical examination heart disease, in ≥1 relative 7. Disability from heart disease in a close relative <50 years of age b) Not prudent to recommend routine use of such tests as 8. Specific knowledge of certain cardiac conditions in family members: hypertrophic or ECG, Echo or graded exercise test dilated cardiomyopathy, long-QT syndrome or other ion channelopathies, Marfan syndrome, or clinically important arrhythmias  Large number of competitive athletes in U.S. Physical examination  Relatively low frequency of life-threatening cardiovascular lesions 9. Heart murmur - supine and standing 10. Femoral pulses to exclude aortic coarctation  Low rate of sudden cardiac death 11. Physical stigmata of Marfan syndrome 12. Brachial artery blood pressure (sitting position) – both arms June 18, 8 2018 2

AHA RECOMMENDATIONS ON AHA CONCLUSIONS ON SCREENING SCREENING  Personal and family history and physical examination for known cardiovascular risk lesions is the best available and most  Not intended to discourage population screening practical approach to screening, regardless of age.  Concern re: false-positive test results  Such screening is an obtainable objective and should be  unnecessary anxiety among athletes and families mandatory for all athletes.  We recommend... history and a physical examination in  unjustified exclusion from life insurance coverage and athletic organized high school (grades 9 through 12) and collegiate competition sports. Screening should then be repeated every 2 years.  Estimate that false-positives exceed true-positive results. EVALUATION INADEQUATE ALTERNATIVE OPINIONS  European Society of Cardiology (ESC) and the International Olympic Committee (IOC) recommend ECG screening in all athletes .  So why did the AHA decide otherwise  Cost  2.5 million amateur competitive athletes (high school + college) in U.S.  Yearly ECGs will cost an estimated $2 billion  includes additional tests to evaluate significance of ECG abnormalities ECG screening  False positives would deny opportunity to those at very low risk for cardiovascular events. 3

COMMENTS RE AHA APPROACH Screening athletes is challenging  Argues AGAINST screening – but recommend screening  Difference in opinion on what constitutes screening  AHA recommends the 12-point screen – NO ECG or other investigation  Argue there is physician shortage for ECG and echo screen  but recommend physician examine every athlete  compares physician resources to Italy – but primary care coverage is similar  PCPs per capita 1/1000 in the U.S. vs. 0.9/1,000 in Italy (OECD data 2009)  Italy – 90% ↓ in athlete deaths a�er ECG screen started in 1983 [Corrado 2006]  Athlete death estimates unreliable  based on media reports  Cost analysis outdated/flawed  Overestimates false positive ECGs; abnormals are manageable  Overestimates echo costs - $500 per echo = $250,000 per detection  - Shorter protocols could reduce cost to $25,000 per detection  Ignores recent advances in echo techniques Marek 2010 14 6/18/2018 Corrado 2006 Obvious Anatomic/Functional Wide range + overlap in athlete vs pathology abnormalities are detectable >90% detectable by Echo June 18, 16 2018 15 Image source: https://marciaruns.wordpress.com/tag/athletes-heart/ 6/18/2018 4

HCM WITH SAM KEY FEATURES OF HCM Hypertrophy Outflow Gradients Systolic Anterior Motion CONTRAST IN POOR QUALITY ECHO Athletic hypertrophy 2 years of training REVERSIBLE on 2% of athletes detraining >5 hrs/week SCD Risk assessment 10% of US Football players HCM > 13 mm 12-16 mm Normal >14 F Maron 1995 Maingourd 1990 <12 Mesko 1989 >16 M Obert 1998 Pellicia 1991 June 18, 20 2018 5

Normal range of physiologic LVH Racial Differences • 300 black athletes  947 athletes • >15 mm  None > 16 mm • 3% (n=9)  <2% (n=16) in HCM range (>13 mm) black • 15 rowers – No white • 1 cyclist • All LV>55mm • ECG normal in 9/16 Pellicia 1991 June 18, June 18, Sharma 2002 21 2018 22 2018 Separating HCM from Athlete’s Heart HCM vs Athlete heart Challenging Challenging LVH History Often uncertain • Age • Fam Hx of SCD • Sex • Hx of Heart Dz • Ethnicity • Murmur Over-Dx Over-Dx Mis-Dx Mis-Dx • Size • Isotonic + Isometric • Type of training Potential SCD risk Interrupt training Elimination from competition Family not screened June 18, June 18, 23 2018 24 2018 6

COST EFFECTIVENESS OF ECG EKG; Cardiopulmonary stress  Adding ECG vs. history + physical exam  Costs $88 per athlete  Saves 2.1 life-years per 1,000 athletes Racial differences  Cost-effectiveness $42,900 per life-year saved • T inversion  Adding ECG vs. no screening EKG • Pathologic Qs  Costs $199 per athlete  2.6 life years per 1,000 athletes • ST depression  Cost-effectiveness $74,100 per life-year saved Healthy  Wheeler; Ann Int Medicine 2010  Study of 510 student athletes HCM • Peak VO2<50 CPET  Addition of ECG to history + physical ml/kg/min - HCM  Improved detection of echocardiographically documented cardiac abnormalities from 5 to 10 out of 11 but increased the false-positive result rate from 5.5% to Sharma 2000 16.9%  Baggish; Ann Int Medicine 2010 June 18, 25 2018 ECG CONCERNS Imaging ECG good ECG questionable Validity of adding ECG to Pre- Variability in interpretation – an HCM Athlete Heart participation Screen obstacle to screening  LV cavity NL/small  LV cavity dilated  Meta analysis of 16 papers  138 athletes  7 cardio & 7 sports MD  Represents best clinical practice to • <45 mm • 55-65 mm prevent or reduce the risk of sudden  Corrado, Uberoi, Marek and Seattle cardiac death in athletes. criteria  Diastology Abnormal  Diastology Normal  Significantly improves sensitivity of  7% abnormal by Seattle history and physical examination  LA dilated  LA Dilated  11-14% by other criteria alone;  Seattle reduced abn ECGS; ↑  Has reasonable specificity and  RWT ↑  RWT ↓ agreement excellent negative predictive value; and it is cost-effective.  Variability remains high June 18, Alattar 2015 Berte 2015 27 28 2018 7

SAM & HEMODYNAMICS LVOT gradients REST 6 mmHg REST 90 mmHg EXERCISE 12 mmHg Or BOTH Exercise 150 mmHg 30 Presentation Title and/or Sub Brand Name Here 6/18/2018 THE HOPKINS HEART HYPE EXPERIENCE – HS ATHLETES Sensitivity Specificity p-Value¹  Heart Hype AHA Quest 47.5% 39.5% >0.05  Brief multimodality screen Vs Echo  Questionnaire + cardiac ascultation+ BP + ECG + focused echo AHA Quest 56.5% 40.6% >0.05  High school age athletes (~500 screened) Vs EKG  How accurate is the questionnaire AHA Quest Vs Echo&  14 pt modified AHA questionnaire 57.3% 43.2% >0.05 EKG  554 student athletes Combined  60% male Low sensitivity and specificity for prediction of  Mean age 17±1.5 for boys and 16±1.3 for girls EKG or Echo findings.  All asymptomatic ¹Fisher’s, ‘N-1’ Chi-square and Z- tests for association 8