SUDDEN CARDIAC DEATH IN YOUNG ATHLETES Can the Cardiac Preparticipation Examination Save Lives

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

• Joel Brenner, MD
• Director, Pediatric Cardiology
• Johns Hopkins Hospital

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Wall Street Journal, 6/23/05
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Sudden Cardiovascular Death During Sports
ParticipationGoals

• 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 for sudden death
• 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
• Marons 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 Death Rates young athletes vs non-athletes
Incidence rates (100,000 person-years)
Sudden deaths
Athletes
55
2.3
Non-athletes
245
0.9
Corrado et al. J Am Coll Cardiol 2003 421959-63
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Relative risk of SD Young athletes vs
non-athletes (Veneto region of Italy 1979-1999)
RR 2.5 CI 1.8-3.4 p lt 0.001
Corrado et al. J Am Coll Cardiol 2003 421959-63
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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-athletesThe
Italian Experience
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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 risk2.5)
• 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(Padua1979-19
96)

• 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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Hypertrophic Cardiomyopathy
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Hypertrophic Cardiomyopathy
Dx unexplained LVH
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Hypertrophic Cardiomyopathy
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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
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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 2001151A
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Clinical Characteristics of Athletes Disqualified
for Hypertrophic Cardiomyopathy

• N. 22
• Age 204 yrs
• Sex ( male) 90
• Reason for echo ECG changes (80)
• LV wall Thickness 193 mm
• LV cavity 432 mm
• LVH after detraining unchanged

Corrado D. N Engl J Med 1998 339 364-369
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Marfan syndrome phenotype
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Marfan syndrome phenotype
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Marfan syndrome atypical phenotype
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Bicuspid aortic valve problem similar to Marfan
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Mitral Valve Prolapse
Mitral valve prolapse (MVP) affects up to 5 of
the general population and up to 17 of young
women and girls.
THE PHYSICIAN AND SPORTSMEDICINE - VOL 24 - NO. 7
- JULY 96
J Am Med Wom Assoc 199449(6)192-196
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Mitral Valve Prolapse

• Mitral valve prolapse (MVP) is the most common
  condition of the heart valves. Some studies show
  it affects 6 of all women.

• MVP can affect both men and women however, more
  than 60 of adults with MVP are women.

• The female-to-male ratio is approximately 31.

• One study in college athletes showed a 21
  incidence in women. Occasionally there may be
  mild mitral regurgitation with the prolapse.

THE PHYSICIAN AND SPORTSMEDICINE - VOL 24 - NO. 7
- JULY 96
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Mitral Valve Prolapse
Risk factors for
Sudden Death

• Severe mitral regurgitation
• Severely thickened mitral valve without
  regurgitation
• Increased heart weight
• Reports in the literature also suggest that
  people with evidence of serious ventricular
  arrhythmia, those with repolarization
  abnormalities, and those with convincing clinical
  symptoms of palpitations and/or syncope may have
  an increased risk of sudden death.

. J Am Med Wom Assoc 199449(6)192-196

• Am Heart J 1984107(2) 378-382

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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
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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 110III-694
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Comparison of 2 decades of screening1982-1991 vs
1992-2001
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 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

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Arrhythmogenic Right Ventricular Cardiomyopathy
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PREPARTICIPATION SCREENINGUSOC 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
QUESTIONNAIREPREVIOUS 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
  4-530, 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.

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SUDDEN DEATH IN ATHLETESEXPERIENCE 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 CENTERELITE ATHLETE
PROFILEMEDICAL HISTORY QUESTIONNAIREREVISED
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
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
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Preparticipation Cardiovascular Screening for US
Collegiate Student-AthletesSurvey of 879 NCAA
Schools
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 PediatricsSection 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 PediatricsSection 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-off
• 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
  of 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
AthletesWill Adding an ECG Help?

• Most common
• Hypertrophic Cardiomyopathy--YES
• Congenital coronary artery anomaly--no
• Less common
• Myocarditismost likely
• Aortic rupture (Marfan syndrome)--no
• Mitral valve prolapsenot usually
• Uncommon
• Arrhythmogenic RV Cardiomyopathy--yes
• Atherosclerotic coronary artery disease--no
• Conduction system abnormalities--yes
• Aortic valve stenosis--no

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ECG Abnormalities
Short PR interval- WPW Long QT interval Short QT
interval
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Torsades de Pointe
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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

• Sudden Unexplained Death Heritability and
  Diagnostic Yield of Cardiological and Genetic
  Examination in Surviving Relatives
• Clinical testing identified 17/43 (40) family
  members of victims of SD with an inherited cause
• Rhythm abnormality 12
• Cardiomyopathy 4
• Hypercholesterolemia 1
• Genetic testing confirmed a mutation in 10/17
  (59)

Tan HL, et al. Circulation 2005
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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

• Future problems
• Can ECG/ECHO be added to the screening process
• Screening to include
• Hypertrophic/dilated cardiomyopathy
• Aortic dilatation
• Coronary origin
• RV outflow tract

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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 ATHLETESCan the
Cardiac Pre-participation Examination Save Lives?

• YES
• But not every life at risk.

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USOC TRAINING CENTERELITE ATHLETE
PROFILESummary of current cardiac history review
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USOC TRAINING CENTERELITE 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 CENTERELITE 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 havehigh blood pressure?
• Do you havehigh cholesterol?

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USOC TRAINING CENTERELITE 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 CENTERELITE ATHLETE PROFILE

• Do you haveracing of your heart or skipped
  heartbeats?
• Do you get tired more quickly than your friends
  do during exercise?
• Do you havea heart murmur?
• Do you have a heart arrhythmia?
• Do you have any other history of heart disease?

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USOC TRAINING CENTERELITE ATHLETE PROFILE

• Have you had a severe viral infection (for
  example myocarditis or mononucleosis) within the
  last month?
• Do you haverheumatic 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 ExaminationReferenc
es

• 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 Cardiologyof 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 ExaminationReferenc
es

• 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 ExaminationReferenc
es

• 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.