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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 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
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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.