Cardiac Clearance and Sudden Cardiac Death in Athletes

1. Cardiac Clearance andSudden Cardiac Death in Athletes Mazen Kawji, MD

2. Disclosures • I have nothing to disclose

3. First…do no harm “I wouldn't ever set out to hurt anyone deliberately unless it was, you know, important — like a league game or something.” Dick Butkus

4. Outline • Epidemiology • Etiology • Athlete’s Heart • Pre-participation Physicals • Additional Testing • Common Red Flags • Causes of Sudden Cardiac Death • 26th Bethesda Conference Guidelines for Athletic Participation

5. Epidemiology • College and Professional Athletes • 500,000 participants each year • Competitive Athletics: • “Several million high school students participate in competitive athletics each year in the United States”. • ‘Other’ Organized Sports Participation • 25 million children and young adults

6. Epidemiology • Incidence of Sudden Cardiac Death: • Organized High School/College Athletes • 1:134,000/Year (Male) (7.47:million/Year) • 1:750,000/Year (Female) (1.33/million/Year) • Air Force Recruits • 1:735,000/Year • Marathon Runners • 1:50,000 Race Finishers (Mean Age 37yo) • In brief, ~ 300 deaths/year. • But the media attention and legal implications, make these events standout.

7. HCM – 36% Coronary Anomalies 17% Increased Cardiac Mass (possible HCM) 10% Ruptured Aorta/Dissect 5% Tunneled LAD 5% Aortic Stenosis 5% Myocarditis 3% Dilated CM 3% Idiopathic Myocdardial scarring 3% Arrhythmogenic RV dysplasia 3% OTHERS… MVP CAD ASD Brugada Syndrome Commotio Cordis Complete heart block QT prolongation syndrome Ebstein’s anomaly Marfan’s Syndrome Wolff-Parkinson White Syndrome – WPW Ruptured AVM SAH Etiology based on largest US data set

8. When in Rome….. • Arrhythmogenic RV dysplasia (22%) is the most common cause of SCD in athletes.

9. Screening requirements • In the US competitive athletes are screened by means of history and physical examination. • Only Europe mandates a resting ECG. • In 1982 the incidence of SCD in Italy was 4.2/100,000 athletes. In 2004 the incidence of SCD decreased markedly to 0.9/100,000. Due to Arrhythmogenic RV dysplasia.

10. Maron BJ et al, JAMA 1996 ; 276 : 199 - 203 Sports at time of death

11. Pre-Participation Physicals • History • Screen for medications and drugs of abuse that can have potential cardiotoxic effects (Beta agonists, Theophylline, TCA’s, Macrolides, Pseudoephedriine, Phenypropanolamine, Tobacco, Alcohol, Cocaine, Amphetamines, Ephedrine, and Anabolic Steroids) • Questions to ask…************************ • Have you ever passed out during or after exercise? • Have you ever been dizzy during or after exercise? • Have you ever had chest pain during or after exercise? • Do you get tired more quickly than your friends do during exercise? • Have you ever had racing of your heart or skipped heart beats?

12. Pre-Participation Physicals • Yes, more questions • Have you had high blood pressure or high cholesterol? • Have you ever been told you have a heart murmur? • Has any family member or relative died of heart problems or sudden death before age 50? • Have you had a severe viral infection within the last month (ie. Myocarditis or mononucleosis) • Has a physician ever denied or restricted your participation in sports for any heart problems?

13. Pre-Participation Physicals – Cont’d • Physical Exam • Gen: physical appearance • ie – Marfan’s Syndrome

14. Pre-Participation Physicals – Cont’d • Physical Exam • Vitals: • BP: Elevated readings confirmed • Proper technique • Pulse: Rate of rise, Contour, Volume, consistency • Normal • Pulsus Bisferiens – Seen in AS, Aortic regurge, HCM - Coarctation of aorta – ie. HTN in arms, but weak femoral pulses AND/OR femoral pulse lags behind that of the radial artery

15. Pre-Participation Physicals – Cont’d • Standing/Squatting: STANDING decreases venous return and reduces the intensity of innocent murmurs (as well as BAD murmurs of AS). • BUT, …STANDING accentuates the murmur of obstructive hypertrophic cardiomyopathy! • Squatting will DECREASE the intensity of the murmur of obstructive hypertrophic cardiomyopathy. • Therefore, the cardiac exam on athletes first supine, then seated, then standing.

16. Pre-Participation Physicals – Cont’d • Indications for echo: • All Diastolic Murmurs • Holosystolic murmurs • Murmurs Grade 3/6 and above • Any murmur that examiner isn’t sure about…ie. CYA? • Features of “Innocent Murmurs”: • Low in intensity and midsystolic in timing, normal splitting, normal DYNAMIC auscultation, absence of a specific pattern of radiation, asymptomatic.

17. Additional Testing American Heart Assoc. Guidelines: exercise ECG screening test men > 40-45 years of age women > 50-55 years of age (or postmenopausal) with 1 independent coronary risk factor hypercholesterolemia or dyslipidemia including low HDL systemic hypertension current or recent cigarette smoking diabetes mellitus a history of myocardial infarction or SCD in a first-degree relative aged < 60 years.

18. Additional Testing • EKG’s • Findings in Athletes considered WNL • Sinus Bradycardia – as low as 30-40 bpm • Various A/V blocks occur in up to 33% of athletes • First Degree (PR>0.2) – Most Common • Second Degree (Mobitz-1 or Wenkeback) • Increased R or S wave voltage without Left axis deviation, QRS prolongation, or LAE • U-waves with up-sloping ST segments and normal T waves • Incomplete RBBB

19. Athlete’s Heart • Endurance and Isometric sporting activities cause structural remodeling and increase in cardiac mass (physiologic hypertrophy). • Increased volume of ventricular chambers • Increased size of L atrium and L ventricular wall thickness • Vary according to sport • Extreme changes reported in Crew, XC skiing, Cycling, Swimming • However, systolic/diastolic fxn is maintained • Occurs in M>F with size related to lean body mass. • May be 2’ to genetics • The amount of exercised-induced LVH in endurance athletes associated with ACE genotype.

20. EKG’s Additional Testing

21. Symptoms • In a recent autopsy study in young military recruits in the US • Army with SCD in relation to exercise • about half of the deceased • recruits complained of premortem symptoms.

22. Quick abbreviations • ARVD = arrhythmogenic right ventricular dysplasia • AS = aortic stenosis • CAA = coronary artery anomoly • DC = dilated cardiomyopathy • HB = heart block • LQTS = long QT syndrome • MC = myocarditis • MVP = mitral valve prolapse • NMS = neurally mediated syncope • TCA = tunneled coronary artery • VP = ventricular preexcitation

23. Exertional Syncope • CV Causes • CAA, LQTS, HCM, MC, DC, AS, WPW, NMS, HB • Additional Testing Needed • EKG, Echo, Exercise Stress Testing - 64 slice CT scan? for CAA

24. Exertional Chest Pain or dyspnea • CV Causes • HCM, CAA, Marfan’s, TCA, MVP, MC, ARVD, AS

25. Palpitations • CV Causes • WPW, LQTS, MVP • Non-CV Causes • Hyperthyroidism, Supplements, Stimulant meds

26. Causes of Sudden Death • Hypertrophic Cardiomyopathy********************** • Sporatic or inherited (autosomal-dominant) • Can predispose to malignant ventricular arrhythmias leading to syncope or sudden death • S/S: • Dyspnea (initially exertional in onset), Angina, Exertional syncope, exertional presyncope, fatigue, palpitations • Exam: • Systolic murmur that increases with valsalva • Testing: • CXR: cardiomegaly • EKG: LVH • Echo: confirmation of HCM • Tx: • B-Blockers • ICD • Septal artery ethanol ablation

27. ECG of HOCM patient

28. Causes of Sudden Death • Coronary Artery Anomalies • In one review of 78 cases of CAA who died of sudden death, 62% of those were asymptomatic • S/S: Only ~ 1/3 of pts have any symptoms of exertional syncope (<25yo) or exertional cp (25-50yo) • Exam: usually normal • Testing: • EKG: usually normal or Q-waves showing infarction • Tx: Immediate exclusion from ALL participation in competitive sports, may need surgical intervention +/- usual tx for MI.

29. Anatomy

30. Commotio Cordis • Traumatic cause of sudden death via arrhythmia (usually v-fib) • Caused by blunt force trauma to chest occurring during the vulnerable repolarization period ( usually on the T-wave and can be the QRS period also) • Some evidence support cardiac injury, but the etiology and electrophysiology have yet to be completely defined

31. Commotio Cordis cont’d • Most commonly seen in adolescent baseball players but also unprotected karate kicks to chest, ice hockey, etc. • Chest protectors and softer core baseballs decrease, but do not eliminate the risk

32. ARVD • Arrhythmogenic Right Ventricular Dysplasia, also known as arrhythmogenic right ventricular cardiomyopathy, is characterized by replacement of the right ventricular muscle by fatty and fibrous tissue. • arrhythmias of right ventricular origin that range from isolated premature ventricular beats to nonsustained or sustained VT and ventricular fibrillation.

33. ARVD cont. • Global or regional right ventricular dysfunction, and late evolution to right or biventricular heart failure. • Incomplete or complete RBBB • Inverted T waves in the anterior precordial leads • Localized prolongation of the QRS complex in leads V1 and V2 • Epsilon waves visible as sharp discrete deflections at the terminal portion of the QRS complex in the anterior precordial leads • Use QRS width in Lead I which is always <120ms • Lead III R>S • S wave upstroke in V1 - V3 >55ms was found in 95 percent of ARVD********

34. ARVD examples, look at V1 - V3 also

35. 26th Bethesda Conference Guidelines for Athletic Participation************* Common Board Exam Topic

36. References • AAFP – Sports Medicine: Strategies for Treating Athletes. Breckinridge, CO. 2004. Francis O’Conner, MD. “Sudden Cardiac Death and Arrhythmias in Athletes” • Beckerman J, Wang P, Hlatky M. Cardiovascular Screening of Athletes. Clin J Sport Med. 2004;Vol 14, Number 3:127-133. • Mellion, Walsh, et al. Team Physician’s Handbook. 3rd edition. Hanley & Belfus; 2002. • Maron, B. Sudden Death in Young Athletes. NEJM. 2003; Vol 349, Number 11:1064-1075. • Pelliccia A, Maron B, et al. Remodeling of left ventricular hypertrophy in elite athletes after long-term deconditioning. Circulation 2002;105:944-949.