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SUDDEN CARDIAC DEATH IN YOUNG ATHLETES Can the Cardiac Pre-participation Examination Save Lives? Joel Brenner, MD Director, Pediatric Cardiology Johns Hopkins Hospital Wall S treet Journal, 6/ 23/ 05 Sudden Cardiovascular Death During


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SUDDEN CARDIAC DEATH IN YOUNG ATHLETES Can the Cardiac Pre-participation Examination Save Lives?

Joel Brenner, MD Director, Pediatric Cardiology Johns Hopkins Hospital

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Wall S treet Journal, 6/ 23/ 05

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Sudden Cardiovascular Death During Sports Participation: Goals

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

  • The young, competitive athlete represents

the popular ideal of cardiac fitness and well- being

  • The sudden death of a well-trained athlete

tends to be well-publicized, and often poorly understood

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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 (?)

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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

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Marc Vivien Foe (Camerun)

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Sudden deaths

Incidence rates (100,000 person-years)

Athletes Non-athletes 55 245 2.3 0.9

Sudden Death Rates: young athletes vs non-athletes

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

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0,5 1 1,5 2 2,5 3 3,5 4 SD per 100,000 person-years Athletes Non-athletes

RR = 2.5

CI = 1.8-3.4 p < 0.001

Relative risk of SD Young athletes vs non-athletes

(Veneto region of Italy; 1979-1999)

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

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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

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Causes of SD in Athletes vs Non-athletes: The Italian Experience

Cause Athletes (N=55) Nonathletes (N=245) Total (N=300) Arrhythmogenic RV CM 12 (22%) 25 (10%)* 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%)

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0.1 0.2 0.3 0.4 0.5 0.6 SD per 100,000 athletes A R V D C A D C C A M V P M y

  • c

a r d i t i s C

  • n

d u c t i

  • n

s y s t e m d i s .

Athletes Non-athletes

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

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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

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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

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Prevalence of HCM in young white people

ECG ECHO

ECG: 0.07% (22 of 33,735) ECHO: 0.10% (2 of 2,030)

Corrado D. NEJM, 1998 Maron B. Circulation, 1995

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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

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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

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Clinical Characteristics of Athletes Disqualified for Hypertrophic Cardiomyopathy N.: 22 Age: 20±4 yrs Sex (% male): 90 Reason for echo: ECG changes (80%) LV wall Thickness: 19±3 mm LV cavity: 43±2 mm LVH after detraining: unchanged

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

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  • Systematic exposure of the athletic young

population to preparticipation screening successfully identified and disqualified athletes with HCM and prevented sudden death

Sudden Death in Young Competitive Athletes

Corrado et al N Engl J Med 1998; 339: 364-369

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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

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Cost per year of life saved

Parameters Hx & Physical Exam 12-Lead ECG

(∈ 30)

Hx & Physical Exam

(∈ 20)

Specificity

91% 95%

Cost to screen 33,735 athl.

∈ 1,012,050 ∈ 674,700

Cost to evaluate abnormal findings in 33,735 athl.

∈ 211,120 ∈ 125,440

Total cost to screen/ evaluate 33,735 athl.

∈ 1,223,170 ∈ 754,990

Number of athl. with HCM identified at screening

43 10 (77% less sensitive)

Cost for each correct diagnosis

28,450 75,500

Cost per year of life saved*

∈ 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

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Comparison of 2 decades of screening

1982-1991 vs 1992-2001*

Causes of disqualification Time interval

___________________________________________________

1982-1991 1992-2001

P value

Cardiovascular diseases 421 388

ns

ARVC 2 (0.5%) 13 (3.3%) 0.003

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

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ARVC and Sudden Cardiac Death

  • ARVC has been discovered only 20 years ago

and for a long time it was either underdiagnosed

  • r 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

  • f Italy and this is expected to result in further

reduction of athletic field deaths

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PREPARTICIPATION SCREENING: USOC POLICY

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

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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)

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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

  • f continuous CPR.

– Autopsy done, results not known.

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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
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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
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Preparticipation Cardiovascular Screening for US Collegiate Student-Athletes

26% Total 14% Division III n=337 30% Division I n=286 Adequate (>9/12) Recommended Elements

Pfister GC. JAMA 2000

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

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

5% 44 Formal CV training 7% 58 Routine non-invasive testing 51% 446 Required yearly 19% 164 Off-campus 81% 719 On-campus 97% 855 Formal screening

Prister GC 2000. JAMA

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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

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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.

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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.

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The Oregon Preparticipation Protocol, 2000

  • Detailed family medical history with parent sign-
  • ff
  • 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

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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

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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

  • f coronary artery, MVP)

– Acquired/familial coronary disease (ASCVD) – Acquired/inflammatory heart disease (myocarditis)

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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

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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

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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

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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

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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?

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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

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SUDDEN CARDIAC DEATH IN YOUNG ATHLETES

Can the Cardiac Pre-participation Examination Save Lives?

YES

But not every life at risk.

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

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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?

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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?
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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?

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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?

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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?
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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?

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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.

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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): 199- 204.

  • 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.

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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: 1597- 1599.

  • Wingfield K. Preparticipation Evaluation: An Evidence-

Based 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.