SUDDEN CARDIAC DEATH IN YOUNG ATHLETES Can the Cardiac Pre-participation Examination Save Lives? Joel Brenner, MD Director, Pediatric Cardiology Johns Hopkins Hospital

Wall Street Journal, 6/23/05

Sudden Cardiovascular Death During Sports Participation: Goals • Prevent the event • Prevent death due to the event

Sudden Cardiovascular Death During Sports Participation • The young, competitive athlete represents the popular ideal of cardiac fitness and wellbeing • The sudden death of a well-trained athlete tends to be well-publicized, and often poorly understood

Rate of sudden death during sports participation in the U.S. is not known • No central registry – Maron estimates 250-300 deaths/year

• Unclear number of sports participants – 7 million high school athletes – 400,000 NCAA athletes – -5 million recreational athletes (?)

Rate of sudden cardiac death during sports participation in the U.S. is not known Generally accepted U.S. estimate is 0.5-2.0/100,000 Maron’s estimate of 300 deaths/year would require an at risk population of 15,000,000 sports participants to result in a death rate of 2/100,000 Italian experience in a fixed geographic area with known number of 12-35 year old sports participants is 2.1/100, 000

Marc Vivien Foe (Camerun)

Sudden Death Rates: young athletes vs non-athletes Sudden Incidence rates (100,000 deaths person-years)

Athletes

55

2.3

Non-athletes

245

0.9

Corrado et al. J Am Coll Cardiol 2003; 42:1959-63

Relative risk of SD Young athletes vs non-athletes (Veneto region of Italy; 1979-1999) SD per 100,000 person-years

4 3,5

RR = 2.5 CI = 1.8-3.4 p < 0.001

3 2,5 2

Athletes Non-athletes

1,5 1 0,5 0

Corrado et al. J Am Coll Cardiol 2003; 42:1959-63

Causes of Sudden Cardiac Death in Young Competitive Athletes in the U.S. Most common: Hypertrophic Cardiomyopathy Congenital coronary artery anomaly Less common: Myocarditis Aortic rupture (Marfan syndrome) Mitral valve prolapse Uncommon: Arrhythmogenic RV Cardiomyopathy Atherosclerotic coronary artery disease Conduction system abnormalities Aortic valve stenosis

Causes of SD in Athletes vs Non-athletes: The Italian Experience Cause Arrhythmogenic RV CM

Athletes (N=55) 12 (22%)

Nonathletes (N=245) 25 (10%)*

Total (N=300) 37 (12%)

Atherosclerotic CAD

10 (18%)

48 (19%)

58 (19%)

Anomalous CA origin

7 (12%)

1 (0.4%)*

8 (3%)

Myocarditis

5 (9%)

27(11%)

32 (11%)

Mitral valve prolapse

6 (11%)

21 (8%)

27 (9%)

Conduction system dis.

4 (7%)

21 (8%)

25 (8%)

Hypertrophic CM

1 (2%)

22 (9%)

23 (7.5%)

Aortic rupture

1(2%)

11(5%)

12(4%)

Dilated CM

1(2%)

10(4%)

11(4%)

Other

8 (20%)

59 (24%)

67 (22%)

Athletes Non-athletes

0.5 0.4 0.3 0.2 0.1

M C yo on ca du rd ct iti io s n sy st em di s.

M V P

C C A

C A D

A R V D

0

Corrado et al. J Am Coll Cardiol 2003; 42:1959-63

SD per 100,000 athletes

0.6

Sudden Death in Young Competitive Athletes • Sport activity in adolescent and young adults is associated with an increase in the risk of sudden death (relative risk=2.5) • Given the substrate of underlying cardiovascular disease such as congenital coronary anomaly, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, and premature coronary atherosclerosis, strenuous physical activity may trigger life-threatening ventricular arrhythmias • Therefore, every effort should be made to recognize the cardiac abnormalities implicated in sudden death during preparticipation screening examination

Preparticipation Athletic Screening (Padua:1979-1996) • Athletes screened: 33,735 • Athletes disqualified: 1,058 (3%) • Cardiovascular causes of disqualification: 621 (59%) • Hypertrophic Cardiomyopathy: 22 (0.07% of 33,735) Corrado et al. N Engl J Med 1998; 339: 364-9

Prevalence of HCM in young white people ECHO ECG

ECG: 0.07% (22 of 33,735) Corrado D. NEJM, 1998

ECHO: 0.10% (2 of 2,030) Maron B. Circulation, 1995

Sensitivity of 12-lead ECG in SD victims of HCM 78 SD victims of HCM

53 Prior 12-lead ECG 51/53 (96%) Positive ECG (LVH, ST-T changes, q waves)

Maron B. Circulation 1982; 65: 1388-94

Sensitivity of preparticipation screening for the detection of patient with HCM at risk for SD Negative History, Physical exam, & ECG 4,469

No HCM by Echo

Pelliccia A & Maron BJ - JACC 2001;151A

Clinical Characteristics of Athletes Disqualified for Hypertrophic Cardiomyopathy N.: Age: Sex (% male): Reason for echo: (80%) LV wall Thickness: LV cavity: LVH after detraining:

22 20±4 yrs 90 ECG changes 19±3 mm 43±2 mm unchanged

Corrado D. N Engl J Med 1998; 339: 364-369

Sudden Death in Young Competitive Athletes

• Systematic exposure of the athletic young population to preparticipation screening successfully identified and disqualified athletes with HCM and prevented sudden death Corrado et al N Engl J Med 1998; 339: 364-369

Screening of young athletes for Hypertrophic Cardiomyopathy Athletes screened 33,735 Positive findings 3,016 (9%) HCM diagnosis by echo 22 (0.07%) Corrado et al. Circulation 2004; 110:III-694

Cost per year of life saved Parameters Specificity Cost to screen 33,735 athl. Cost to evaluate abnormal findings in 33,735 athl. Total cost to screen/ evaluate 33,735 athl. Number of athl. with HCM identified at screening Cost for each correct diagnosis Cost per year of life saved*

Hx & Physical Exam Hx & Physical 12-Lead ECG Exam (∈ 30) (∈ 20)

91%

95%

∈ 1,012,050

∈ 674,700

∈ 211,120

∈ 125,440

∈ 1,223,170

∈ 754,990

43

10 (77% less sensitive)

28,450

75,500

∈ 14,220

∈ 37,750

*Based on the assumption that 10% of affected athletes identifierd and disqualified by both PPS modalities willl live an additional 20 years

Comparison of 2 decades of screening 1982-1991 vs 1992-2001* Causes of disqualification Cardiovascular diseases ARVC

Time interval

P 1992-2001 value

___________________________________________________

1982-1991 421

388

ns

2 (0.5%)

13 (3.3%)

0.003

*Center for Sports Medicine, National Health Service, Padova, Italy

ARVC and Sudden Cardiac Death • ARVC has been discovered only 20 years ago and for a long time it was either underdiagnosed or regarded with skepticism by the medical community • In the last 10 years, with increased awareness of clinical findings suggestive of ARVC more and more athletes are now being identified by preparticipation screening in the Veneto Region of Italy and this is expected to result in further reduction of athletic field deaths

PREPARTICIPATION SCREENING: USOC POLICY WITH SPECIAL THANKS TO ED RYAN Director, Division of Sports Medicine USOC, Colorado Springs, CO, USA

U.S.OLYMPIC TRAINING CENTER MEDICAL HISTORY QUESTIONNAIRE PREVIOUS FORMAT

• 2 page health survey • 3 questions potentially regarding cardiovascular integrity – Have you ever had a seizure? – Have you ever been told you have epilepsy? – Do you have … heart disease? (murmur, rheumatic fever, stenosis)

SUDDEN DEATH IN ATHLETES: USOC EXPERIENCE • 18 yo male boxer, DOD 2/25/90 – Passed routine pre-fight physical exam between 4-5:30, 2/25/90. – Went out to jog on track with teammate. Jogged several laps, complained of chest pain. Continued to jog, collapsed. CPR begun. 911 called. EMT response in 5 minutes, defib in ambulance, died after 45 minutes of continuous CPR. – Autopsy done, results not known.

SUDDEN DEATH IN ATHLETES: EXPERIENCE OF USOC • 13 yo male gymnast, DOD 10/11/01 – Finished routine on pommel horse – Complained of shortness of breath, staggered, collapsed, seized. CPR unsuccessful. – Past history of fainting while on high bar – Autopsy negative • Presumed arrhythmia • Family counseled to seek medical evaluation

USOC TRAINING CENTER ELITE ATHLETE PROFILE MEDICAL HISTORY QUESTIONNAIRE REVISED FORMAT • 6 page health survey, lifestyle inquiry, medication/drug use survey • 21 questions related to cardiac concerns

Preparticipation Cardiovascular Screening for US Collegiate Student-Athletes

Division I

n=286

Division III

n=337

Total

Adequate (>9/12) Recommended Elements 30% 14% 26%

40% of screening forms omitted questions related to exertional chest pain, dyspnea, fatigue, familial heart disease, premature sudden death, Marfan syndrome Pfister GC. JAMA 2000

Preparticipation Cardiovascular Screening for US Collegiate Student-Athletes Survey of 879 NCAA Schools Formal screening

855

97%

On-campus Off-campus Required yearly

719 164 446

81% 19% 51%

Routine non-invasive testing Formal CV training

58

7%

44

5%

Prister GC 2000. JAMA

Preparticipation Screening of Student Athletes in US High Schools • All 50 states formally required PPE, but 8 had no official questionnaire to guide examiners • 0-56% of forms contained specific CV risk factor questions • Only 5-37% of forms included specific maneuvers directed toward identifying CV disease • BP measurements were not included in 86% of forms • None of the 50 states offered standard qualifications for examiners, 25 sanctioned non-physician examiners • 40% of state high school associations did not offer standardized PPE forms complying with AHA recommendations or had no screening requirement Wingfield K. Clin J Sport Med 2004

American Academy of Pediatrics Section on Sports Medicine and Fitness • SCREENING EXAMINATION – Before participating in any sports, young athletes should have a complete physical exam that includes a detailed personal and family history of any heart conditions. – Exam should be done by a health care provider with the training, medical skills, and background to recognize heart disease.

American Academy of Pediatrics Section on Sports Medicine and Fitness • Electrocardiography and echocardiography are not recommended as part of regular screening of athletes. This is because a heart problem is found very rarely.

The Oregon Preparticipation Protocol, 2000 • Detailed family medical history with parent signoff • Physical exam by health care professional trained in CV risk identification, in a quiet room – Auscultation should be performed sitting, supine and squatting using the diaphragm and the bell of a stethoscope • Comment about S1, S2, ejection click, murmurs, femoral pulses

The Oregon Preparticipation Protocol, 2000

• Targeted use of 3 non-invasive tests – ECG or stress ECG – Hand-held 2D echo and color flow study – Cardiac MRI for suspected risk of coronary artery malformation

Sudden Cardiac Death in Young Athletes • Underlying cardiac risk can be divided in to: – Genetic/familial structural abnormalities (HCM, DCM, ARVC, Marfan/CT abnormality) – Genetic/familial conduction abnormalities (long QT syndrome, other channelopathies) – Isolated anatomic abnormalities (anomalous origin of coronary artery, MVP) – Acquired/familial coronary disease (ASCVD) – Acquired/inflammatory heart disease (myocarditis)

Sudden Cardiac Death in Young Athletes • Little data is available on the current state of the PPE in the US • The evidence for the efficacy of mass screening in the US is conflicting • The PPE is unevenly administered – Lack of standardized questionnaire – Variable quality of cardiac evaluation – Volunteer projects using echo are not likely to be sustainable for the general population of student athletes

Causes of Sudden Cardiac Death in Young Athletes—Will Adding an ECG Help? Most common: Hypertrophic Cardiomyopathy--YES Congenital coronary artery anomaly--no Less common: Myocarditis—most likely Aortic rupture (Marfan syndrome)--no Mitral valve prolapse—not usually Uncommon: Arrhythmogenic RV Cardiomyopathy--yes Atherosclerotic coronary artery disease--no Conduction system abnormalities--yes Aortic valve stenosis--no

Sudden Cardiac Death in Young Athletes • Legal considerations • In Knapp v. Northwestern University, federal appellate court recognized the value of recommendations and guidelines to determine reasonable levels of athletic participation for persons with cardiovascular abnormalities • Liability issues in screening evaluations need to be clearly established • Recommendations for follow-up care/evaluation need to be tracked • Decision-making for participation needs to be based on available medical information for the health benefit of the individual, independent of the needs of the team

Sudden Cardiac Death in Young Athletes • Future goals • The variability in the PPE questionnaire must be eliminated Role of national organizations, such as AAP. AHA, AASM, athletic trainers, and others, to promote standardization

• The variability of the cardiac component of the physical examination must be minimized Feasibility of specific cardiac retraining for all examiners Role of digital acquisition of heart sounds and central analysis providing odds ratios of cardiac abnormality to guide more extensive cardiac evaluation

Sudden Cardiac Death in Young Athletes • Future problems • Can ECG/ECHO be added to the screening process • Organization of systematic screening of 7 million high school athletes poses enormous logistic issues • Increased expense of testing and timely reading of studies— in the U.S., who will pay?

Sudden Cardiac Death in Young Athletes • Additional evaluation of estimated 9-10% false positive subjects is probably a larger expense than the initial population screening • Use of detailed ECHO, stress testing, ultrafast CT or MRI scans to define coronary anatomy will return most of this group to sports participation • Evaluation must be timely, if the student athlete is to return to full sports participation

SUDDEN CARDIAC DEATH IN YOUNG ATHLETES

Can the Cardiac Pre-participation Examination Save Lives?

YES But not every life at risk.

USOC TRAINING CENTER ELITE ATHLETE PROFILE Summary of current cardiac history review

USOC TRAINING CENTER ELITE ATHLETE PROFILE • Do you ever have chest tightness? • Does running ever cause chest tightness? • Have you ever had chest tightness, cough, wheezing, asthma….which made it difficult for you to perform in sports?

USOC TRAINING CENTER ELITE ATHLETE PROFILE • Have you ever had a seizure? • Have you ever been told that you have epilepsy? • Have you ever been told to give up sports because of health problems? • Do you have…high blood pressure? • Do you have…high cholesterol?

USOC TRAINING CENTER ELITE ATHLETE PROFILE • Do you have trouble breathing or do you cough during or after activity? • Have you ever been dizzy during or after exercise? • Have you ever fainted or passed out when exercising? • Have you ever had chest pain during or after exercise?

USOC TRAINING CENTER ELITE ATHLETE PROFILE • Do you have…racing of your heart or skipped heartbeats? • Do you get tired more quickly than your friends do during exercise? • Do you have…a heart murmur? • Do you have a heart arrhythmia? • Do you have any other history of heart disease?

USOC TRAINING CENTER ELITE ATHLETE PROFILE • Have you had a severe viral infection (for example myocarditis or mononucleosis) within the last month? • Do you have…rheumatic fever?

USOC TRAINING CENTER ELITE ATHLETE PROFILE • INQUIRY RELATED TO FAMILY HISTORY – Has anyone in your family under age 50 died suddenly? – Do you have a family history of heart disease?

The Cardiac Pre-participation Examination References •

•

•

International Olympic Committee Medical Commission: Sudden cardiovascular death in sport: Lausanne Recommendations. www.olympic.org Maron BJ, et al: Cardiovascular preparticipation screening of competitive athletes: a statement for health care professionals from the sudden death committee (clinical cardiology) and congenital cardiac defects committee ( cardiovascular disease in the young), American Heart Association 1996: 94 (4): 850-856. Study Group of Sport Cardiology…of the European Society of Cardiology: Cardiovascular preparticipation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. Eur Heart J 2005: 26 (5): 516-524.

The Cardiac Pre-participation Examination References • •

•

• •

Maron BJ. How should we screen competitive athletes for cardiovascular disease? Eur H J 2005; 26 (5): 428-430. Corrado D, et al. Does sports activity enhance the risk of sudden death in adolescents and young adults? J Am Coll Cardiol 2003: 42 (11): 1959-1963. Maron BJ, et al. Sudden death in young competitive athletes: clinical, demographic, and pathological profiles. JAMA 1996; 276 (3): 199204. Van Camp SP, et al. Nontraumatic sports deaths in high school and college athletes. Med Sci Sports Exerc 1995; 27 (5): 641-647. AAFP, AAP, AMSSM, AOSSM,AOASM: Preparticipation Physical Evaluation, ed 3. McGraw-Hill, 2004.

The Cardiac Pre-participation Examination References • Bader S. Risk of sudden cardiac death in young athletes: which screening strategies are appropriate? Ped Cl NA: 51, 5, Oct, 2004. • Fister GC. Preparticipation cardiovascular screening for US collegiate student-athletes. JAMA 2000; 283: 15971599. • Wingfield K. Preparticipation Evaluation: An EvidenceBased Review. Clin J Sport Med 2004; 14: 109-122. • Fuller C. Cost effectiveness analysis of screening of high school athletes for risk of sudden cardiac death. Med Sci Sports Exerc 2000; 32 (5): 887-890.