1. THE HEART OF THE ATHLETE WILLIAM B BAKER MD FACC

2. EXERCISE IS GOOD FOR YOU

3. BENEFITS OF EXERCISE DISEASE PREVENTION Cardiovascular Diabetes Osteoporosis, joint health FITNESS WEIGHT CONTROL ENJOYMENT Personal Goals Competition

4. DEFINITIONS FOR THIS TALK EXERCISE: Any form of physical activity, done on a regular basis, with the purpose of achieving a specific goal Low level to vigorous Recreational (including “play”) to competative ATHLETE: Anyone who is exercising YOUNG ATHLETE: Less than 35 years old ADULT ATHLETE: Greater than 35 years old

5. COULD YOUR “WORKOUT” CAUSE YOU CARDIOVASCULAR HARM? ANSWER: YES THE RISK IS SMALL THE CONSEQUENCES ARE SIGNIFICANT WHAT THE RISK IS AND WHAT CONDITIONS ARE RESPONSIBLE FOR THE RISK VARY BY AGE

6. Who are we talking about, what are the numbers and what are we talking about

7. THE YOUNG ATHLETE AND THE RISK (US numbers) All deaths related to exercise: 120/year (excluding trauma) Deaths caused by CVD: < 100/year Approximately 1 CVD death/100,000/year CVD death in affected athletes is not limited to exercise times At least 2-3 X more likely during exercise All the “conditions” that might harm athletes are just as prevalent in non-athletes. Athletes are at higher risk.

8. THE YOUNG ATHLETE HOW MANY ARE AT RISK? 44 Million youths participate in “sports programs” 3.5 Million high school athletes 500,000 college athletes in the US 10,000 “pro-athletes” in the US IF A CONDITION THAT CAN HARM AN ATHLETE AFFECTS 1 IN 500 YOUTHS, HOW MANY ARE AT RISK?

9. THE ADULT ATHLETE Harder to define the numbers and risk Heart disease is common among adults Exercise programs vary No organized reporting program Marathoners: <1/100,000 on race day Tri-athletes: 1.5/100,000 on race day Recreational runners: 1/10,000/year or 1/396,000 hours of running Nordic Skiers: 1/607,000 hours Individuals with disease are 2 -3-X more likely to have an event during exertion.

10. THE YOUNG ATHLETE A SAMPLING OF THE CAUSES Structural Heart Disease Hypertrophic Cardiomyopathy Anomalous Origin of the Coronary Arteries Arrhythmogenic Right Ventricular Cardiomyopathy Myocarditis/Cardiomyopathy Valvular Disease The “Channelopathies” Marfan Syndrome

11. THE ADULT ATHLETE A SAMPLING OF THE CAUSES Coronary Artery Disease Valvular Heart Disease Cardiomyopathy “Young Athlete” Disease

12. THE YOUNG ATHLETE

13. THE YOUNG ATHLETE and SUDDEN CARDIAC DEATH Rare events Without warning Devastating Occur in healthy individuals Attract attention

14. MECHANISM OF SUDDEN DEATH Ventricular Tachycardia and Ventricular Fibrillation Normal EKG Ventricular Tachycardia Polymorphic Ventricular Tachycardia Ventricular Fibrillation

15. THE YOUNG ATHLETE and SUDDEN CARDIAC DEATH The “Underlying Substrate”: Many of these conditions predispose to lethal arrhythmia There can be changes in the athlete’s heart that may increase the risk Hypertrophy (the “muscular heart”) LV and RV dilation (the “enlarged heart”) Increased demand and “adrenalin”

16. THE YOUNG ATHLETE SPECIFIC EXAMPLES (An incomplete review)

17. HANK GATHERS 1967 - 1990

18. HYPERTROPHIC CARDIOMYOPATHY

19. Affects 1 in 500 individuals Genetically determined Sporadic or inherited At least 11 genes, 1400 mutations Accounts for 35 – 40% of athletic deaths Can be symptomatic/detectable before SCA Increased risk with age Ventricular arrhythmia is primary cause of death

20. HYPERTROPHIC CARDIOMYOPATHY Treatment: Medical Treatment: Implantable Defibrillator “Disqualified” from participation in in all but low effort sports (bowling, curling) regardless of symptoms, phenotype, treatment.

21. The IMPLANTIBLE CARDIAC DEFIBRILLATOR (ICD)

22. DISQUALIFIED? The 36 th Bethesda Conference

23. THE 36 TH BETHESDA CONFERENCE

24. ANOMALOUS ORIGIN OF THE CORONARY ARTERIES

25. Accounts for 15 – 20% of sudden death in young athletes Can be symptomatic (< 50%) Chest discomfort Shortness of breath Palpitations Fainting Treatment: Medical or Surgical May be “cleared” to participate if corrected

26. ARRYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

27. ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY Prevalence: 1/1000 – 2000 Genetic, 30% inherited. Accounts for 5% of sudden death in young athletes Can be symptomatic: palpitations, fainting Treatment: medical, ICD Disqualified from competitive sports

28. MYOCARDITIS/CARDIOMYOPATHY

29. Accounts for 5 -10% of sudden cardiac arrests in young athletes Causes: “viral”, inherited/genetic, idiopathic Can be symptomatic: shortness of breath, palpitations, fatigue/weakness, fainting, chest discomfort Treatment: Medical, time, ICD, transplant Disqualified from most competitive sports. May return if recover. ICD = no contact sports

30. MARFAN SYNDROME Connective tissue disorder Genetic 25% sporadic Autosomal Dominant 1/3000 – 5000

31. MARFAN SYNDROME

32. COMMOTIO CORDIS Vulnerable moment High force, specific area Baseball, hockey, karate Kids more vulnerable 20% survival Boys > girls Prevention key Training to avoid impact ? vests

33. INHERITED ARRHYTHMIA and SUDDEN CARDIAC ARREST The “Channelopathies”

34. WHAT IS A CHANNEL?

35. THE CHANNELOPATHIES AND SUDDEN CARDIAC ARREST ( A SAMPLING ) Long QT Syndrome Brugada Syndrome Catecholaminergic Polymorphic Ventricular Tachycardia Short QT

36. THE CHANNELOPATHIES AND SUDDEN CARDIAC ARREST Inherited/genetic conditions Lead to Ventricular Tachycardia and Ventricular Fibrillation Evident (variably/intermittently) on EKG Cause of Sudden Cardiac Arrest in both athletes and non-athletes. Exercise does increase the risk in many of these conditions

37. THE CHANNELOPATHIES THE LONG QT SYNDROME

38. THE CHANNELOPATHIES: LONG QT Not rare: 3000 – 4000 deaths/y in children/adolescents Inherited/genetic 12 types/genes, hundreds of different mutations Variable “lethality” AR associated with deafness Variable expression Acquired form Medications/drugs Electrolyte changes Increased risk of SCA with exercise, risk variable based on type SCA in athletes: not rare, numbers not clear EKG +, gene +, symptom + : Disqualified from competitive sports

39. ACQUIRED LONG QT Medications: www.qtdrugs.orgwww.qtdrugs.org Antiarrhythmics Antibiotics: Levaquin, Zithromax (Z pack), erythromycin Antidepressants: Tricyclics, Prozac, Celexa Tamoxifen diuretics 140 other drugs Methadone Combinations of drugs Electrolytes: Low K+, Mg++, Ca++ Genetic + Drugs, ? Unmasked congenital form Reversible

40. ACQUIRED LONG QT AND EXERCISE ? Drug + exercise interaction ? Electrolyte changes with exercise Dehydration Excessive “free water” intake Losses with sweating Diuretics ? Greater risk with endurance events The Perfect Storm: Congenital substrate + drugs + exercise

41. THE CANNELOPATHIES BRUGADA SYNDROME Genetic Genetic testing variable Na+ channel EKG variable Provocative testing Multiple types Male > Female Avg age at DX: 41 Fever/hyperthermia trigger Night time trigger Treatment: ICD, limited medications Caution advised for competitive sports with no history of events With history of events or ICD low level sports only

42. THE CHANNELOPATHIES: CATECHOLAMINERGIC POLYMORPHIC VT

43. CPVT Genetic, at least 2 gene mutations Inherited Emotional and physical triggers. Symptoms: dizziness and syncope Usually presents in childhood and adolescence Treatment: Medical therapy, ICD + medical, Sympathectomy, Medical therapy for gene + asymptomatic. Generally recommend against competitive sports, ICD precludes contact sports

44. OTHER ARRHYTHMIA WOLFF PARKINSON WHITE 1/400 Often Incidental finding Can present with symptoms Often first diagnosed in adulthood Risk of V-fibrillation Risk stratify asymptomatic Pts Ablation OK to participate in competitive sports once treated

45. SCREENING YOUNG ATHLETES Recommendations vary widely internationally and within the US Recommendations vary widely based on level of participation Not clear if definitely reduces risk Findings variable with time Variable age of onset These are relatively rare diseases Needs to be done regularly until adult age

46. SCREENING GOAL To identify those at risk Prevent injury and lethal events TO ASSIST YOUNG ATHLETES AND THEIR FAMILIES IN MAKING RATIONAL DECISIONS REGARDING THE RISK OF ATHLETIC PARTICIPATION

47. THE PREPARTICIPATION EXAM Review for symptoms Dizziness or fainting, shortness of breath, palpitations, chest discomfort, can’t keep up Family History Premature death “Death under unusual circumstances” Physical exam Murmurs, build, pulses

48. WHAT ABOUT EKGs Not recommended routinely in US Required in Europe Controversial Not clear it helps Athletes often have EKG changes that are “normal” False negatives, False positives Cost of EKGs, Cost of additional testing, Cost of disqualifying athletes Estimated $80,000 to find one case

49. LOWERING RISK IN THE YOUNG ATHLETE Preparticipation Exam Parental involvement in children and adolescents Coaches/trainer/athlete awareness Symptom awareness Workout/practice design Hydration/electrolyte replacement AEDs in close proximity when feasible and AED training CPR training of coaches/trainers/athletes

50. SCREENING RELATIVES “BACKWARD AND FORWARD” Can both include exclude disease

51. THE ADULT ATHLETE CARDIOVASCULAR DISEASE IS THE PRIMARY CAUSE OF DEATH IN ADULT ATHLETES

52. THE ADULT ATHLETE Primary Cause: Coronary Artery Disease Cardiomyopathy Vascular Disease Arrhythmia Valvular Heart Disease

53. THE ADULT ATHLETE The adult athlete can still have almost any of the conditions of the young athlete.

54. CORONARY ARTERY DISEASE STILL NUMBER ONE

55. JIM FIXX 1932 - 1984

56. WHAT IS THE RISK? 800,000 Heart attacks/year 400,000 Sudden Cardiac Death Sudden Death: First symptom in 50% 2 – 3 X as likely to suffer a cardiac event during exercise in those with disease Numbers during exercise unknown Marathon Risk: 1/50,000 – 100,000/race 2012: 550,000 finished a marathon 2011: 500,00 started their first marathon Nobody is keeping track outside of organized events Odds are there are many cardiac events during unorganized exercise that are not reported

57. WHAT IS THE RISK OF EXERCISE? Relatively infrequent Not rare Unexpected No prior history of cardiac disease Healthy Devastating for “victims”, families, communities

58. DETERMINANTS OF EXERCISE RISK Probability of Cardiac Disease Intensity and Duration of Exercise RISK INCREASES WITH INCREASED RISK OF UNDERLYING CVD, INTENSITY, DURATION

59. MEASURING INTENSITY The Metabolic Equivalent or MET 3.5 ml O2/kg/min

60. MET 1.Sitting……………………………………………….1.0 2.Walking at 2.5 m/h……………………………2.9 3.Biking at 10 m/h……………………………….4.0 4.Elliptical……………………………………………5.5 5.Jogging…………………………………………….7.0 6.Swimming (moderate)……………………..8.0 7.Swimming (hard)…………………………….12.0 8.Running 8 min mile…………………………12.5 9.Bike Racing (not drafting) > 20m/h….16.0

61. RATING OF PERCEIVED EXERTION RPE

62. EXERCISE INTENSITY Light Daily activities, gentle walk RPE < 3, < 3 METs Moderate Brisk walk, easy jog or bike RPE 3 – 5, < 6 METs “Talk Sing” Vigorous/Intense Running, Biking, High Intensity Interval, “Boot Camp” RPE 7 – 10, METs > 6 Fail “Talk Sing”

63. EXERCISE DURATION Dehydration Electrolyte changes Increased inflammation Hyperthermia Pushing through Most cardiac events during marathons occur past the 22.5 mile marker

64. RECOMMENDED DURATION (health and fitness goal) American Heart Association 150 min/week of moderate exercise 75 min/week of vigorous exercise OK to break it up

65. WHAT IS CORONARY ARTERY DISEASE? HOW DO I CATCH IT?

66. CARDIOVASCULAR DISEASE THE PRIMARY CULPRIT

67. FACTORS INCREASING THE LIKLIHOOD OF CORONARY ARTERY DISEASE Age High Blood Pressure Abnormal Cholesterol Values Family History Smoking

68. FACTORS INCREASING THE LIKLIHOOD OF CORONARY ARTERY DISEASE NON-TRADITIONAL Cholesterol variants Lp(a) Particle size Genetic Vascular physiology/metabolism Inflammation

69. GLOBAL RISK THE GREATER THE NUMBER OF RISK FACTORS, THE GREATER THE RISK

70. THE HEART ATTACK

71. ISCHEMIA Increased HR Increased Contractility Increased Inflammation Electrolyte Changes Dehydration Demand > Supply

72. ISCHEMIA AND SCD DEMAND > SUPPLYISCHEMIA CHEST PAIN SOB PERFORMANCE NON-LETHAL ARRYTHMIA LETHAL ARRHYTHMIA

73. WHAT’S THE TREATMENT PREVENTION PREVENTION PREVENTION MEDICAL REVASCULARIZATION

75. OTHER POTENTIAL LETHAL CARDIAC DISEASE AND EXERCISE DILATED CARDIOMYOPATHY HYPERTROPHIC CARDIOMYOPATY

76. OTHER POTENTIAL LETHAL CARDIAC DISEASE AND EXERCISE AORTIC DISSECTION Risk Factors: ASCVD, especially hypertension Sporadic, associated with aneurysm, genetic Sheer force Increased risk with high static component exercise

77. OTHER POTENTIAL LETHAL CARDIAC DISEASE AND EXERCISE VALVULAR HEART DISEASE Aortic stenosis Aortic insufficiency Mitral Valve Prolapse

78. NONLETHAL ARRHYTHMIA ATRIAL FIBRILLATION SUPRAVENTRICULAR TACHYCARDIA

79. EXERCISE AND NONLETHAL ARRHYTHMIA European Heart Journal 2014 52,000 Nordic Skiers Mean age: 38 Twice the risk of non-athletes Higher risk with more races Higher risk with faster times Mechanism: ? inflammation

80. DETERMINING YOUR RISK AGE CVD RISK FACTORS HISTORY OF EXERCISE SYMPTOMS FAMILY HISTORY OF SUDDEN DEATH, FAINTING, ARRHYTHMIA, DEATH UNDER UNUSUAL CIRCUMSTANCES CORONARY ARTERY CALCIUM SCORING

82. WHO NEEDS SCREENING Everyone over 20 years old should know their risk factors for CVD and periodically re- evaluate them Everyone should discuss with their physician their exercise routine (Physicians rarely ask) A “baseline” history and exam is indicated as part of the risk evaluation for exercise It is reasonable for adults to have at least one baseline EKG

83. RISK LEVEL Low Risk: man < 45, woman < 55, no CVD risk factors, no symptoms, no worrisome history Moderate Risk: man > 45, woman > 55, 1 or 2 CVD risk factors (not DM) High Risk: History or Symptoms of CVD, DM, age > 65, > 2 CVD risk factors

84. RISK LEVEL LOW MODERATE HIGH HIGH MOD LOW INTENSITY WHO NEEDS PRE-EXERCISE TESTING

85. Match your type, intensity and duration of exercise to your goal

86. IF YOU ARE EXERCISING DON’T IGNORE SYMPTOMS WHETHER WITH EXERCISE OR NOT Decreased performance Chest discomfort Shortness of breath Irregular heart beats / palpitations Dizziness or fainting

87. CONCLUSIONS EXERCISE IS GOOD FOR YOU EVERYBODY SHOULD EXERCISE EXERCISE CARRIES A SMALL RISK OF A CARDIAC EVENT THAT IS “AGE” SPECIFIC GET APPROPRIATE “SCREENING” DON’T IGNORE SYMPTOMS. THERE IS NO LIFETIME WARRANTY FROM A SINGLE SCREENING