Sudden Cardiac Death
causes and screening
in college athletics
Matthew Pecci, MD
Assistant Professor in Family Medicine
Director of Sports Medicine Boston University
Objectives
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• Scope of the problem
• Review the most common causes of SCD in the US
• Review current consensus on preparticipation(PPE)
screening
• Review the pros and cons of the addition of ECG
screening to the PPE exam
SUDDEN DEATH
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• Sudden death in athletics can occur from direct or
indirect causes
• direct - traumatic
• indirect - atraumatic
SUDDEN DEATH
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• Most indirect causes are cardiac in origin.
• A very small number of indirect causes of sudden
death are noncardiac
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heat stroke
CVA
pulmonary embolus
drug abuse
SUDDEN DEATH
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• From 1931 to 1986 direct causes of sudden death
outnumbered indirect cause by 2 to 1.
• From 1982 to the present this has reversed with
indirect causes now outnumbering direct causes by 2
to 1.
SUDDEN CARDIAC DEATH
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• Sudden cardiac death (SCD) in athletes:
• nontraumatic, nonviolent, unexpected death due to cardiac causes
within one hour after the onset of symptoms, and occurring within
one hour of sports participation
SCD EPIDEMIOLOGY
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How prevalent a problem is SCD?
SCD Incidence
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• Incidence of SCD in US is unknown
• Studies to date have relied on surveys, and nonmandatory reporting systems that may underestimate the
true incidence
• There has been some push from the sports medicine
community for a national registry
• National Center for Catastrophic Sports Injury
• US National Registry of Sudden Death in Athletes
SCD Incidence
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• Van Camp et al
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SCD in HS and college athletes 1983-1993
Identified by media reports and newspaper clippings
100 cases of SCD
Estimated number of athletes based on HS and college participation
rates
• SCD rate 1:300,000 annually
1.
Van Camp SP, Bloor CM, Mueller FO, Cantu RC, Olson HG. Nontraumatic sports death in high school and
college athletes. Med Sci Sports Exerc. 1995;27:641– 647.
SCD Incidence
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• Maron et al
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1.
Minnesota HS athletes
Mandatory insurance for catastrophic death in HS athletes
12 year period, grades 10-12
3 cases of SCD occurred during study period
Rate of SCD was 1:200,000 participants annually
Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden cardiac death during competitive sports activities in
Minnesota high school athletes. J Am Coll Cardiol. 1998;32:1881–1884.
SCD Incidence
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• Maron et al
• SCD over 27 year period in athletes up to age 39
• Identified cases of SCD through various sources – news media,
internet, direct reports, etc
• 1866 cases identified, in 1353 cause of death determined
• Rate of SCD 1:164,000 participants annually
1.
Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes:
analysis of 1866 deaths in the United States, 1980–2006. Circulation. 2009;119:1085–1092.
SCD Incidence
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• Eckart et al
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SCD in military recruits over 25 year period
Ages 18-35
Mandatory reporting of all deaths
Autopsy confirmation of cause
Rate of SCD 1:9,000 recruit-years
Eckart RE, Scoville SL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits.
Ann Intern Med. 2004;141(11):829–834
SCD Incidence
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• Harmon et al
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SCD in NCAA student-athletes over 5 year period
Identified by NCAA database, media reports, and insurance claims
273 death with 45 SCD
Incidence SCD 1:43,770 participants per year
Breakdown by sport, sex, and race
Div 1 men’s basketball SCD rate 1:3100 per year
Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of Sudden Cardiac Death in National Collegiate
Athletic Association Athletes. Circulation. 2011;123:1594-1600.
SCD Incidence
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• Incidence likely somewhere in between
• Based on available data experts estimate the true
incidence of SCD in the US is 1 in 75,000
SCD
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What is the most common cause of SCD in
athletes?
SCD
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• Over age 35 - 90% of SCD are due to coronary artery
disease
• In many cases the athlete has no known history of
disease
• Acute plaque rupture
SCD
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• Cause of SCD in athletes less than 35yrs
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36-50% hypertrophic cardiomyopathy
10-19% coronary artery anomalies
6% myocarditis
5% ruptured aorta (Marfans syndrome)
<5% aortic stenosis, right ventricular dysplasia, CAD, Ion channel
disorders
HCM
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• Hypertrophic cardiomyopathy
• Clinically recognized in 1 in 500 in the general
population (0.2%)
• Genes mutations on at least 12 genes which code
components of the heart muscle fibers
• Autosomal dominant with variable expression
• Myocardial disarray, myocardial scarring
HCM
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• Classic findings:
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LV wall thickness of 16mm or more (nl < 12mm)
vast disarray of the walls affected and extent of hypertrophy
septum to free wall ratio >1.3
Small LV cavity size < 45mm with impaired diastolic function
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HCM
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• Functional findings:
• impaired filling of the LV during diastole due to small cavity size
• abnormal flow across the mitral valve
• pressure gradient to blood flowing into the aorta due to thickened
septum
HCM
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• Incidence of SCD in HCM
• 2-4% / year in adults
• 4-6% / year in children and adolescents
HCM
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• Causes of SCD in HCM - speculative
• Arrythmia - ventricular tachyarrythmias
• hypotension from diastolic dysfunction and outflow tract obstruction
• myocardial ischemia
Screening for HCM
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• Can we identify HCM in an athlete before it causes
SCD?
• Family history – autosomal dominant
• symptoms - syncope, CP, fatigue, dyspnea
• physical exam findings
HCM
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• Most athletes are asymptomatic and their sentinel
event is SCD
• Estimated that only 21% of athletes with HCM have
signs or symptoms1
1. Maron BJ, Shirani J, et al. Sudden death in young competitive athletes. Clinical,
demographic, and pathological profiles. JAMA. 1996;276(3):199–204
HCM
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• Physical exam - murmur from outflow obs.
• Harsh, mid systolic
• heard best at right upper sternal border
• decreases in intensity with increased blood flow to the heart i.e.
squatting, lying down
• increase in intensity with decreased blood flow to the heart i.e.
Valsalva, standing
• Estimated that only 25% of HCM patients have a
murmur
Is an ECG helpful?
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• An ECG will be abnormal in 75-95% of patients with
HCM
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wide array of abnormalities
prominent Q waves
deep negative T waves
increased QRS voltage associated with ST depression or T-wave
inversion.
Is an echocardiogram helpful?
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• Echocardiography remains the standard to confirm the
diagnosis of HCM
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identifying pathologic LV wall thickness (>16 mm)
a non-dilated LV with impaired diastolic function
Septum to free wall ratio > 1.3
Changes may not be apparent until physical maturity
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HCM diagnosis uncertainty
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• In cases where the diagnosis of HCM is uncertain (ie,
borderline LV wall thickness)
• Cardiac magnetic resonance imaging (MRI)
• Genetic testing
• Repeat echocardiogram after 4 to 6 weeks deconditioning
• deconditioning should resolve the hypertrophy in an athletic heart
and may help in distinguishing from HCM.
HCM and participation
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• No single clinical, morphologic, or electrophysiologic
feature has been found to be a reliable predictor of
SCD in patients with HCM
• 36th Bethesda Conference:
• “present recommendations for sports eligibility remain conservative
and homogeneous for those athletes within the diverse HCM clinical
spectrum”
• “Broad recommendation to exclude such individuals from competitive
sports will, by definition, deny participation to some unnecessarily”
Maron BJ, Zipes DP, et al. 36th Bethesda conference. Journal of the American College
of Cardiology. 2005;45(8):1317-75.
HCM and participation
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• 36th Bethesda recommendations:
“Athletes with a probable or unequivocal clinical diagnosis of
HCM should be excluded from most competitive sports, with
the possible exception of those of low intensity (class IA).
This recommendation is independent of age, gender, and
phenotypic appearance, and does not differ for those
athletes with or without symptoms, LV outflow obstruction, or
prior treatment with drugs or major interventions with
surgery, alcohol septal ablation, pacemaker, or implantable
defibrillator.”
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Coronary Artery Anomalies
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• The right and left coronary arteries arise from the
aortic sinuses and are responsible for blood supply to
the heart
• a small percentage of people have arteries which
originate in atypical locations
• certain of these atypical arterial originations may affect
blood flow to the heart
Coronary Artery Anomalies
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• Most common anomaly leading to SCD:
• left coronary from the right sinus of valsalva - causes a severe
angle in the artery which disrupts blood flow
• Others:
• hypoplastic coronary arteries
• tunneled epicardial arteries
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Coronary Artery Anomalies
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Cause of death is from myocardial ischemia
Very difficult to detect with screening
No genetic component
No exam findings
Small percentage may have symptoms - review of 78
cases - 62% asymptomatic
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Questions?
SCREENING for SCD
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• No outcome based study exists on the effectiveness of
the PPE
• The American Heart Association recommends a
detailed history and exam consisting of 12 items1
• The 4th edition PPE monograph endorses the AHA
recommendations2
1.
Maron BJ, Thompson PD, et al. Recommendations and considerations related to
preparticipation examination screening for cardiovascular abnormalities in
competitive athletes: a 2007 update. Circulation. 2007;115(12):1643-55.
2.
American Academy of Family Physicians , American Academy of Pediatrics ,
American College of Sports Medicine , et al. Preparticipation Physical Evaluation.
In: Roberts W, Bernhardt D editor. 4th edition. Elk Grove (IL): American Academy of
Pediatrics; 2010;
SCREENING for SCD
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• Personal History:
• Exertional chest pain
• Unexplained syncope or near syncope
• Excessive exertional and unexplained dyspnea/fatigue associated
with exercise
• Prior recognition of a heart murmur
• Elevated systolic blood pressure
SCREENING for SCD
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• Family History:
• Premature death(sudden and unexpected, or otherwise) before age
50 years due to heart disease, in 1 or more relatives
• Disability from heart disease in a close relative <50 years of age
• Specific knowledge of certain cardiac conditions in family members:
hypertrophic or dilated cardiomyopathy, long QT syndrome or other
ion channelopathies, Marfan syndrome, or clinically important
arrhythmias
SCREENING for SCD
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• Additional questions in PPE monograph:
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Does your heart ever race or skip beats?
Has your doctor ever ordered tests for your heart?
Have you ever had an unexplained seizure?
Do you get more tired or short of breath more quickly than your
friends during exercise?
• Family history of unexplained or unexpected death prior to age 50
including drowning, unexplained car accidents, SIDs
• Family member with a pacemaker or implantable defibrillator
• Family member with unexplained seizures, fainting or near drowning
SCREENING for SCD
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• Physical exam:
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Auscultation for a murmur (sitting and standing)
Femoral pulses to exclude aortic coarctation
Stigmata of Marfans syndrome
Brachial artery BP (sitting)
SCREENING for SCD
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• AHA recommendation compliance:
• In 2000 only 26% of NCAA schools had forms containing at least 9 of
the 12 items
• In 2005 48 states had approved forms for high schools and 39 of
these forms had at least 9 items
• In 2010 a survey to the Washington chapter of AAP, AAFP, and high
school ADs only 6% of providers and 0% of schools were compliant.
Only 47% of physicians and 6% of ADs reported awareness of the
guidelines.1
Madsen NL, Drezner JA, Salerno JC. Sudden death screening in adolescent
athletes: an evaluation of compliance with national guidelines. Br J Sports Med 2013;
47:172-77
SCREENING for SCD
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• What should colleges do?
• Make sure forms contain recommended history and exam items
• If you are performing your own PPEs make sure you have qualified
examiners who are aware of the guidelines
• If you are not performing your own PPEs make sure forms are
adequately reviewed and if there are any questions athletes
reexamined
ECG Screening in the USA
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• 2007 consensus statement by the American Heart
Association recommended not to screen athletes with
an ECG
• “Admirable proposal deserving serious consideration”, “many
epidemiologic, social, economic, and other issues” which impact this
screening proposal in the US
• The 4th edition Pre-participation examination
monograph does not recommend for or against ECG
screening
ECG and the Italian Data
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• Mandatory screening of competitive athletes with
history, physical and ECG begun in 1982
• Population-based observational study
• Incidence of SCD in athletes in prescreening (197981), early screening (1982-92), and late screening
(1993-2004); causes of SCD
• Incidence of SCD in athletes decreased by 89%, 10
fold decrease in deaths from cardiomyopathies
Corrado D, Basso C, Pavei A, et al. Trends in sudden cardiovascular death in young competitive
athletes after implementation of a preparticipation screening program. JAMA. 2006; 296:1593–
1601.
ECG and the Italian Data
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• Based on the Corrado study the European Society of
Cardiology and the International Olympic Committee
now recommend ECG screening as part of the Preparticipation exam
Pelliccia A, Fagard R. Bjørnstad HH, et al. A European Society of Cardiology consensus
document: recommendations for competitive sports participation in athletes with cardiovascular
disease. Eur Heart J. 2005;26:1422–1445.
No ECG Rationale
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Italian data
Scope of problem in US
ECG Specificity
Cost
Challenge of mass screening
Ethical considerations for testing
Italian Data
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• Not controlled
• Mandatory H+P, and ECG all implemented in 1982
• High prescreening annual death rate in athletes of
3.6/100,000 person years somewhat accounts for the
large change with screening
• Lowest death rate 0.4 death/100,000 person years is
similar to reported death rates of HS and college
athletes in US without the use of ECG screening
Italian Data
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• 44% of the disqualifications were from high blood
pressure and valvular heart disease which presumably
can be identified without an ECG
• Unclear from data how many of the cardiomyopathies
were diagnosed from history vs exam vs ECG
• Decreased in death rate from cardiomyopathies during
the study period was mainly due to ARVC not HCM
• 24 of the 55 athletes who died during the study period
had 1 or more positive findings (FH, palpitations,
syncope, chest pain, murmur, or ECG findings)
Scope of problem in US
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• Unknown
• No national reporting system – we don’t know the
numbers of cases of SCD or how many competitive
athletes in US at any given time
• Presumed low incidence
Specificity of ECG
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• 7% false positive rate in Italian study with ECG
analyzed by sports cardiologists
• Studies in US using ECG at PPEs report 10%-25%
abnormal ECGs requiring further evaluation
• Study at Harvard over 3 years adding ECGs to
screening had 16.9% false positive rate1
1.Baggish AL, Hutter AM, et al. Cardiovascular screening in college athletes with
and without electrocardiogram. Ann Intern Med 2010;152:269-75
Cost of ECG Screening
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• Cost projection model in US based on the Italian data:
• Annual cost of $2.5 to $3.4 billion dollars
• Estimates 4,813 lives saved over a 20 year period
• $10.4 to $14.4 million per life saved
Halkin A, Steinvil A, et al. Preventing sudden death of athletes with electrocardiographic
screening:what is the absolute benefit and how much will it cost? J Am Coll Cardiol.
2012;60(22):2271-76
Cost of ECG Screening
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• 5 year ECG screening program at the University of
Virginia:
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Screened 1,473 athletes over 5 years
5 significant cardiac abnormalities found(one long QT, no HCM)
ECG abnormal in 275 resulting in 359 additional tests
Cost of history and physical screening $343,725
Adding ECG cost and testing cost additional $551,145
Malhotra R, West JJ, et al. Cost and yield of adding electrocardiography to
history and physical screening Division I intercollegiate athletes: a 5-year
experience. Heart Rhythm. 2011;8(5):721-7.
Cost of ECG Screening
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• 10 million middle and high school athletes would be
eligible for screening annually
• $25 H+P, $50 for ECG then assume 15% need further evaluation
including cardiologist visit $100, echo $400
• Minimum annual cost of 2 billion dollars which does not factor into the
administrative costs of such a large scale program
Large scale US screening
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• Difficult to make consensus recommendations if there
is no consistent infrastructure for implementation
• Lack of widespread sports medicine infrastructure in
both high schools and colleges in US:
• Untrained examiners more false positives
• Selective or unequal implementation based on socioeconomic
status or profile of college/sport
• Social and medico legal consequences
Large scale US screening
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• Survey study of NCAA Div. I Football programs:
• 35 of 74 schools or 47% use non-invasive screening
• Most use ECG but 17 also use echocardiograms
• 30% utilize a cardiologist for these screenings
• Would the response be the same for non-revenue
sports or other division schools?
Coris EE, Sahebzamani F, et al. Preparticipation cardiovascular screening among
National Collegiate Athletic Association Division I Institutions. Br J Sports Med.
2013;47:182-4.
Ethical Consideration
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• 879 athletes(30 cases of HCM) disqualified in the
Italian study, none died of SCD at end of study period.
• Did they assume a sedentary lifestyle or were they low risk for
SCD anyway?
• At least 3 cases were reported to have committed suicide
• We do not know what characteristics of HCM make
SCD more likely – “deny participation unnecessarily”
• Do we want to commit athletes who may not be at risk
for SCD to Class IA activities for the rest of their lives?
Conclusions for No ECG in US
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• Incidence of SCD is low overall
• Informed decision requires more information:
• Need a national SCD registry – athletes, symptoms,
screened
• Data on specificity of revised criteria in untrained
• Cost control measures
• More info on the natural history of HCM
• Controlled trials prior to widespread recommendation
Bottom Line: ECG use at your school
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• Will pick up more asymptomatic and potentially lethal
cardiac conditions
• Need infrastructure to obtain ECGs, and trained
interpreters
• Need to accept consequences of more false positives:
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Cost of further work up
Time for further work up
Potential disqualification of equivocal cases
Legal consequences of disqualification
Conclusions
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• Difficult to identify the scope of the problem in the US
• The most common causes of SCD in college aged
athletes in the US specifically HCM
• AHA and PPE monograph recommendations for
history and physical screening – is your school
compliant?
• ECG controversy
• Know how to respond to non traumatic syncope in the
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athlete - ABCs with initiation of CPR, immediate call
for help, be familiar with the use of an AED
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Thank You!
Myocarditis
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• Acute inflammation of the myocardium which can cause arrythmia
and SCD
• Coxsackie virus B believed to cause 50% of cases
• Others viruses echovirus, adenovirus, influenza, Chlamydia
• Present as typical viral illness followed by progressive exercise
intolerance and congestive symptoms
Myocarditis
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• ECG may show diffuse low voltage, ST-T wave changes, heart
block, or ventricular arrhythmias.
• Serologic testing may show leukocytosis, eosinophilia, an
elevated sedimentation rate or C-reactive protein, and increased
myocardial enzymes.
• Echocardiography will confirm the diagnosis within the right
clinical context showing a dilated LV, global hypokinesis or
segmental wall abnormalities, and decreased LV ejection fraction.
Myocarditis
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Athletes may return to training and competition after a period of about 6
months if:
• LV function, wall motion, and cardiac dimensions return to normal
(based on echocardiographic and/or radionuclide studies at rest and
with exercise)
• clinically relevant arrhythmias such as frequent and/or complex
repetitive forms of ventricular or supraventricular ectopic activity are
absent on ambulatory Holter monitoring and graded exercise testing
• serum markers of inflammation and heart failure have normalized
• the 12-lead ECG has normalized. Persistence of relatively minor
ECG alterations such as some ST-T changes are not, per se, the
basis for restriction from competition.
Marfans Syndrome
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Disorder of connective tissue
prevalence of 1 in 5,000
Genetic
mutation in the gene coding fibrillin
Individuals with Marfans are tall with long limbs i.e.
basketball and volleyball athletes
Marfans Syndrome
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• Body systems affected:
• Skeletal - tall, arachnodactyly, high arched
palate, hyperextensable joints, scoliosis,
chest deformity
• Skin - stria
• Ocular - retinal detachment, myopia, lens
subluxation (60% of cases)
• Pulmonary - spontaneous pneumothorax
Marfans Syndrome
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• CV - ascending aorta dilatation, aortic dissection,
aortic valve insufficiency, mitral valve prolapse or
insufficiency, arrythmias
• CV complications are the major cause of morbidity and
mortality
Marfans Syndrome
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• Exam findings:
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Arm span > height
Reduced upper to lower body ratio
pectus deformity
hyperextensable joints
murmurs - AI, MI, MVP
skin - stria
Marfans Syndrome
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• Ghent criteria – clinical features highly suspicious for
Marfans
• Diagnosis:
• With + family history - positive features in at least 1 major systems
• With - family history - positive features in at least 2 major systems
Marfans Syndrome
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• All athletes highly suspicious for Marfans syndrome
should have an echocardiogram which shows some
abnormality 95% of the time, and a slit lamp which
demonstrates lens subluxation 60% of the time.
Marfans Syndrome
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• Class IA, or IIA activities if no aortic dilatation, no
moderate aortic regurgitation, and no family history of
dissection or SCD
• Only class IA activities if any of these signs exist