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The Atlantic Rift: Guidelines for Athletic


ScreeningWhere Should Canada Stand?

Article in The Canadian journal of cardiology April 2016


Impact Factor: 3.94 DOI: 10.1016/j.cjca.2016.02.055

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Canadian Journal of Cardiology 32 (2016) 400e406

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The Atlantic Rift: Guidelines for Athletic ScreeningdWhere
Should Canada Stand?
Paul Poirier, MD, PhD, FACC, FAHA,a,b Sanjay Sharma, BSc(Hons), MD, FRCP(UK),c
and Andrew Pipe, CM, MD, LLD(Hon), DSc(Hon)d
a
Institut Universitaire de Cardiologie et de Pneumologie de Que bec, Universite Laval, Que bec City, Que bec, Canada
b
Faculty of Pharmacy, Universite Laval, Que bec City, Que bec, Canada
c
St Georges University of London, London, United Kingdom
d
University of Ottawa Heart Institute, Ottawa, Ontario, Canada

ABSTRACT 
RESUM 
E
Sudden cardiac death (SCD) in a young seemingly healthy athlete is a La mort subite dorigine cardiaque (MSOC) chez les jeunes individus
tragic and often highly publicized event. Preparticipation screening apparemment en sante  est un e ve
nement tragique et souvent trs
aims to identify those affected by cardiovascular diseases who may be publicise. Le de
pistage avant la participation un sport vise de
celer
at higher risk of SCD during sports participation. There are conicting les individus atteints de maladies cardiovasculaires qui sont expose s
recommendations from the American Heart Association and the un risque plus e leve
 de MSOC. Des recommandations contradictoires
European Society of Cardiology regarding screening electrocardio- proviennent de la Socie te
 Americaine de Cardiologie et la Socie  te

grams (ECGs) before participation in sports. The use of an ECG as a Europe enne de Cardiologie concernant les e lectrocardiogrammes de
screening strategy has been questioned, with a large number of depistage (CG) avant la participation. Lutilisation des CG comme
abnormal test results observed in athletes resulting from the electro- gie de de
strate pistage a souvent e te
 remise en question du fait dun
cardiographic changes that occur in a highly trained individual over- grand nombre de re sultats anormaux observe s lors des examens
lapping with ndings suggestive of a pathologic condition. An alise
re s chez des athltes en raison de modications lCG qui

Sudden cardiac death (SCD) is dened as an unexpected therapeutic strategies are available to minimize the risk of
witnessed death of an apparently healthy individual occurring death. The widespread publicity after such a death frequently
within 1 hour of symptom onset or an unwitnessed death includes calls for more effective screening of athletes. In most
occurring within 24 hours. Sudden cardiac death in athletes is instances, however, there are no prodromal warning
the leading cause of nontraumatic death, with a wide range of symptoms, and SCD is the rst manifestation of disease in
incidence reported, from 1 in 3000 in some subpopulations to young athletes,2 in contrast to middle-aged nonathletes in
1 in 1 million. Male persons, blacks, and basketball players whom warning symptoms (chest pain, dyspnea) frequently
seem to be at a higher risk.1 Although rare, such deaths have a occur before SCD.3 Of note, coronary disease events are
devastating impact because athletes are perceived as epito- clustered around the nish line in mass-participation
mizing good health; the sudden unanticipated death of an events.4
athlete precipitates questions and concerns often spurred by
extensive media coverage. These deaths are particularly tragic
because the majority result from inherited or congenital Athletes, Recreational Sports Participants, or
cardiac diseases that are detectable and for which several Weekend Warriors
A competitive athlete is dened as one who participates in
an organized team or individual sport that requires regular
competition against others as a central component, places a
Received for publication December 11, 2015. Accepted February 15, 2016. high premium on excellence and achievement, and requires
Corresponding author: Dr Paul Poirier, Faculty of Pharmacy, Laval some form of systematic (and usually intense) training. The
University, Institut Universitaire de Cardiologie et Pneumologie de Quebec, distinction between competitive athletes and those involved
2725 Chemin Ste-Foy, Quebec City, Quebec G1V 4G5, Canada. Tel.: 1-
418-656-4767; fax: 1-418-656-4581. in recreational sports lies in the ability and freedom of a
E-mail: paul.poirier@criucpq.ulaval.ca participant to judge when it is prudent to reduce or stop
See page 404 for disclosure information. physical exertion.5 Competitive athletes may also be dened

http://dx.doi.org/10.1016/j.cjca.2016.02.055
0828-282X/ 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
Poirier et al. 401
Guidelines for Athletic Screening

abnormal 12-lead ECG triggers further examinations, which are apparaissent chez les individus trs entrane s et qui se superposent
expensive given the low diagnostic yield of most abnormal electro- des resultats e
vocateurs dune pathologie. LGG 12 de rivations qui
cardiographic patterns. Universal screening of young athletes poses est positif entrane dautres examens dispendieux e tant donne le faible
logistic and nancial challenges. There are currently no Canadian rendement diagnostique de la plupart des trace s anormaux dCG. Le
guidelines regarding preparticipation screening of athletes. Screening depistage universel des jeunes athltes pose des difculte s logistiques
of athletes ignores the much larger group of young nonathletes who et nancires. Il nexiste actuellement pas de lignes directrices cana-
participate in vigorous recreational activity and who collectively diennes concernant le de pistage des athltes. Le de pistage des ath-
represent a population in which a much larger number of SCDs can be ltes ne tient pas compte du groupe plus nombreux de jeunes non-
predicted to occur. While waiting for the best screening approach in athltes qui participent des activites re
cre
atives vigoureuses et qui
Canada, increased awareness of and access to automated external representent collectivement une population chez qui lon peut pre dire
debrillators, along with training in cardiopulmonary resuscitation, can la survenue dun nombre beaucoup plus grand de MSOC. Dans lat-
help reduce the number of SCDs. In some jurisdictions, electrocar- tente dune meilleure approche de de pistage au Canada, une sensi-
diographic screening has been eschewed in favour of such an bilisation accrue et un accs aux debrillateurs externes automatiques
approach. Specic physician training in the eld of sports cardiology ainsi quune formation en RCR peuvent aider re duire le nombre de
with availability of experts throughout Canada may be a useful start. MSOC. Certaines autorite s ont rejete
 le depistage par CG en faveur
We provide suggestions and call for the development of Canadian dune telle approche. Une formation particulire des me decins dans le
guidelines by appropriate organizations. domaine de la cardiologie du sport ainsi que la disponibilite dexperts
travers le Canada peut constituer un point de de part utile. Nous don-
nons des suggestions et demandons le laboration de lignes directrices
canadiennes par les organismes approprie s.

as individuals who are engaged in exercise training and prevalence of 3-4 per million population.15,16 Insofar as the
competition on a regular basis, exercising > 10 hours per debate for screening is concerned, the discussion needs to be
week. Elite athletes are a subgroup of this population. Dif- guided by the prevalence of young athletes harboring disease
ferences in characterizing the sporting population likely rather than the prevalence of SCD. Several studies have
contribute to the large variations in the reported incidence of reported that 1 in 300 young athletes and nonathletes are
SCD in sports. Athletes have been found to have a higher risk affected by inherited or cardiac diseases capable of causing an
of SCD compared with nonathletes but not in all studies.6-8 exercise-related SCD. The considerably lower SCD rate
Although deaths among young competitive athletes are higher reects the fact that most diseases implicated in SCD in the
than in their sedentary counterparts, the overall denominator young have low event rates. The lowest rate of SCD among
for all exercising individuals dictates that exercise-related SCD young competitive athletes (0.46 per 100,000 athletes per
occurs much more frequently in recreational athletes.9 academic year) in high school grades 10-12 has been reported
in Minnesota.17

Causes of SCD in Athletes


The causes of SCD in athletes include structural,
arrhythmogenic, and acquired cardiac abnormalities (Table 1). Table 1. Causes of sudden cardiac death in athletes
Some athletes experience syncope, chest pain, dyspnea Inherited: structurally abnormal heart
disproportionate to the physical exertion, seizure, and palpi- Cardiomyopathies
tations before an event.2,10 In athletes  35 years, most events Arrhythmogenic cardiomyopathy, right ventricular cardiomyopathy, or
result from atherosclerotic coronary artery disease,2,11 whereas dysplasia
Dilated cardiomyopathy
in those < 35 years, genetic and acquired cardiovascular Hypertrophic cardiomyopathy, idiopathic left ventricular hypertrophy
abnormalities, particularly cardiomyopathies, are more Left ventricular noncompaction
commonly responsible.12,13 Screening for a family history of Other
premature (< 40 years) cardiovascular disease and SCD Coronary artery abnormalities
Valvular heart disease (bileaet mitral valve prolapse syndrome, bicuspid
should occur at a young age, because most SCDs in young aortic valve)
athletes are caused by inherited cardiac disease.2,14 Aortopathies (eg, ascending aortic aneurysm, Marfan syndrome)
Inherited: structurally normal heart
Channelopathies
Magnitude of the Problem Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia
Sudden death in sports is rare, becoming increasingly Idiopathic ventricular brillation
prevalent with age. A literature review reveals large variations Long QT syndrome
in the incidence of SCD among competitive and recreational Acquired: structurally abnormal heart
athletes. Reported prevalence rates for SCD during sports vary Ischemic heart disease
Myocarditis
and reect methods of data collection, athlete demographics, Exercise-induced arrhythmogenic right ventricular cardiomyopathy
and sporting discipline. Unfortunately, most reports regarding Acquired: structurally normal heart
SCD in athletes are derived from voluntary reporting or the Commotio cordis
media. More detailed analysis of systematic registries such as Substance abuse
the Italian pathology registry in Veneto and the National Environmental factors (eg, hypothermia or hyperthermia, electrolyte
disturbances)
Collegiate Athletic Association Athletes registry reveals a
402 Canadian Journal of Cardiology
Volume 32 2016

Current Practice and Models (United States vs relevant because there is emerging evidence that most sport-
Europe) related SCDs result from sudden arrhythmic death syn-
Although SCD during sports is rare, the societal impact of drome, most commonly caused by ion channel diseases.12,18
such events, loss of decades of life-years, and the ability to Although cardiac imaging studies are the gold standard for
detect and manage the diseases implicated in SCD have identifying cardiomyopathies, it is well recognized that at least
caused both American and European cardiac societies to 90% of patients with hypertrophic cardiomyopathy and 50%
endorse preparticipation cardiovascular screening. Although of patients with arrhythmogenic right ventricular cardiomy-
only 2 countries in the world (Italy and Israel) practice state- opathy have an abnormal ECG. Cardiac screening studies
sponsored screening, most elite sporting organizations in the from Italy,18 the United Kingdom,27 the United States,28
Western world recommend some form of screening of elite Australia,29 and the Netherlands have shown that electrocar-
competitors.18,19 Screening programs may involve all or a diography is superior to screening based solely on a health
combination of the following as preliminary investigations: a questionnaire and physical examination for detecting athletes
health questionnaire inquiring about family and personal with potentially serious cardiac disease.30 In the large 30-year-
history, physical examination, and a 12-lead ECG. Athletes old state-sponsored screening program in Italy, an ECG
with abnormal results are subject to further examinations such detected > 80% of cases of hypertrophic cardiomyopathy.31
as echocardiography, stress testing, 24-hour Holter moni- In a meta-analysis of 15 screening studies in > 45,000 ath-
toring, and cardiac magnetic resonance imaging (MRI) to help letes, an ECG was 5 times more sensitive than a history and
conrm or refute the presence of a potentially serious cardiac 10 times more sensitive than a physical examination and had a
abnormality. The American Heart Association (AHA) and the higher positive likelihood ratio, a lower negative likelihood
European Society of Cardiology (ESC) both advocate ratio, and a lower false-positive rate.32 A total of 160 poten-
screening of elite athletes. However, they differ in that the tially lethal cardiovascular conditions were detected (0.3%).
AHA recommends taking a thorough medical and family The authors suggested that a 12-lead ECG should be
history along with a physical examination, whereas the Eu- considered best practice in screening athletes, whereas the use
ropean Society of Cardiology recommends the addition of a of history and physical alone should be re-evaluated.32 One
12-lead ECG in the initial screening stages.20,21 A recent must remember that the most frequent anomalies found in
AHA/American College of Cardiology (ACC) statement this meta-analysis were Wolff-Parkinson-White syndrome
reafrmed their position,22 noting that there is no compelling (42%), long QT syndrome (11%), and hypertrophic cardio-
reason to conne screening to young competitive athletes and myopathy (11%), which are unlikely to be found by history
exclude nonathletes.23 The American model is cheap and and physical examination alone. The positive predictive value
pragmatic but has poor sensitivity, with values as low as 6%. of most apparently abnormal electrocardiographic patterns is
Most athletes are asymptomatic before SCD, and most dis- low (< 5%); however, deep T-wave inversion in the inferior
eases implicated in SCD during sports are not associated with
physical signs. A family history is often absent even in affected
athletes, because diseases such as hypertrophic cardiomyopa-
thy and long QT syndrome have low event rates; therefore, Table 2. The 14-element American Heart Association
family members may not have presented with a sentinel event. recommendations for preparticipation cardiovascular screening of
In a seminal article, Maron et al.24 described the de- competitive athletes
mographics of 134 young athletes with SCD. Of them, 115 Medical history (parental verication is recommended for high school and
had been screened in accordance with the AHA recommen- middle school athletes)
dations, and a correct diagnosis was identied in only 1 Chest pain/discomfort/tightness/pressure related to exertion
(0.9%). Unexplained syncope/near-syncope judged not to be of neurocardiogenic
Although controversy remains regarding the screening (vasovagal) origin; of particular concern when occurring during or
after physical exertion
ECG, all experts agree that the preparticipation screening Excessive exertional and unexplained dyspnea/fatigue, associated with
should include a cardiovascular-oriented history and physical exercise
examination (Table 2).20 However, the AHA questionnaire Previous recognition of heart murmur
may yield a very high rate of positive responses.25 Approxi- Elevated systemic blood pressure
Previous restriction from participation in sports
mately 25% of athletes screened with AHA personal and Previous testing for the heart ordered by a physician because of family
family history elements may be referred for cardiovascular history
evaluation so that approximately 2.5 million athletes would be Premature death (sudden and unexpected or otherwise) before 50 y of age
referred for further cardiovascular evaluation, in contrast to attributable to heart disease in  1 relative
the extremely low incidence of SCD in athletes. To our Disability because of heart disease in a close relative aged < 50 y
Hypertrophic or dilated cardiomyopathy, long-QT syndrome, or other ion
knowledge, there has been no evaluation of the appropriate- Physical examination
ness of the AHA-14 elements or the Stanford Heart murmur
questionnaire.25,26 Refers to heart murmurs judged likely to be organic and unlikely to be
innocent; auscultation should be performed with the patient in both
the supine and standing positions (or with Valsalva maneuver)
specically to identify murmurs of dynamic left ventricular outow
Efcacy of the ECG tract obstruction
Addition of a 12-lead ECG to the prescreening process has Femoral pulses to exclude aortic coarctation
the potential for detecting athletes with electrophysiological Physical stigmas of Marfan syndrome
cardiac disease, such as the long QT, Brugada, and Wolff- Brachial artery blood pressure (sitting position), preferably taken in both
arms
Parkinson-White syndromes. This may be particularly
Poirier et al. 403
Guidelines for Athletic Screening

or lateral leads, or both, has a relatively high predictive value. compete. The initial estimated prevalence of type 2 changes in
In a recent study of 155 athletes with deep T-wave inversion athletes of 5% has proved to be an underestimate. The data
predominantly in the inferior or lateral leads, or both, 41% of were derived from a nonselect cohort of white athletes and
athletes were diagnosed with cardiomyopathy after echocar- have been associated with unacceptably high-false positive
diography and cardiac MRI testing. Other electrocardio- rates (9%-22%) in elite endurance athletes; in athletes of
graphic patterns highly suggestive of cardiac pathologic African origin, false-positive rates may exceed 40%. When
conditions include ST-segment depression, pathologic Q factors like age, sex, sporting discipline, body habitus, and
waves (Q/S > 0.25), left bundle branch block, and a corrected ethnicity are considered,43 most type 2 electrocardiographic
QT interval  500 ms.33 patterns do not lead to a clear diagnosis after investigation.
The ECG is effective in detecting disease, but there is The ECG is thus a poor tool for discriminating between
debate regarding whether early diagnosis equates to a life cardiac physiology and pathology. More recent criteria have
saved. Data from the mandatory Italian screening program accounted for black ethnicity and provided less conservative
is the best evidence that screening may save lives. In a limits for an abnormal QT interval, which has resulted in a
prospective study using a pathology registry for SCD in the signicant drop in false-positive rates in white athletes to 3%-
Veneto region of Italy, the incidence of SCD among ath- 5%, but there remains an issue with black athletes. Isolated T-
letes screened with electrocardiography fell from 3.6 per wave inversions in leads V1-V4 in black athletes should in
100,000 to 0.4 per 100,000, representing a 90% reduction most cases not prompt further evaluation of asymptomatic
in SCD.18 In contrast, a similar electrocardiography pro- individuals.44-46 The cost of follow-up investigations to
gram in Israel showed no difference in the prevalence of conrm or refute the presence of a serious cardiac disorder and
SCD in athletes when comparing deaths rates 12 years the potential to unfairly disqualify athletes from participating
before screening vs 12 years after screening was imple- in sports are problematic. There are also concerns relating to
mented.19 Determination of the numerator of SCD in the the consistency in the interpretation of preparticipation ECGs
study from Israel relied solely on reports in 2 newspapers; among experienced physicians.47 Even among sports cardiol-
therefore, it is likely that many deaths 12 years before ogists, the variability in interpretation is as high as 20%.48
screening were not detected because media reporting during Electrocardiographic screening is also associated with false-
that period was not as developed. negative results. Diseases of the aorta and coronary arteries
are not usually detectable with electrocardiography and
account for up to 16% of all SCD in young athletes. Similarly,
Concerns Relating to Electrocardiographic a resting ECG will not reveal incomplete expressions of
Screening cardiomyopathy or catecholaminergic polymorphic ventricular
Although the ECG is superior to screening with a health tachycardia. In some jurisdictions, electrocardiographic
questionnaire and physical examination alone, there are screening has not been advocated, but there has been an
several concerns relating to nationwide electrocardiographic increased emphasis on addressing preventable deaths in the
screening, including false-positive results, false-negative re- young by emphasizing debrillator placement and training in
sults, cost, and lack of infrastructure. In Europe, the cost of a cardiopulmonary resuscitation, suicide prevention, and stra-
12-lead ECG is wV10 vs US$39-$47 in the United tegies to reduce motor vehicle accidents.48
States.21,34 The cost per life saved is estimated at $10.6-$14.4 Fundamental to the preparticipation screening debate is
million in the United States.34 Data from the Italian study the balance between (1) lives saved; (2) number of athletes
suggest that 33,000 athletes need to be screened to save 1 life tested; (3) associated psychological, ethical, and legal issues;
at a cost per life saved of $1,320,000.35 This money may be and (4) cost. There is a grey zone between pathologic
better spent in secondary prevention of SCD in sports or conditions and athletic physiological adaptations, producing a
allocated to urgent widespread health care needs like the real clinical challenge.20 Multiple issues were captured in a
increasing rates of obesity in the young.36 recent AHA/ACC publication regarding the 12-lead ECG as
The athletes ECG reects increased vagal tone and a screening test in healthy young individuals.26
increased cardiac chamber size. Some athletes may show
profound repolarization changes that overlap with cardiomy-
opathy or ion channel diseases. Several publications provide Where Should Canada Stand?
electrocardiographic criteria for evaluation of athletes. The There are currently no guidelines regarding pre-
rst electrocardiographic criteria appeared in 1998 and were participation electrocardiographic screening in Canada. In
adapted by the ESC in 2005.21 More recently, a multiple- 2013, the Canadian Academy of Sport and Exercise Medicine
association working group produced the further rened (CASEM) made a global recommendation to screen national-
Seattle Criteria.37 Electrocardiographic patterns have now level, high-performance athletes with electrocardiography.
been recognized that should be classied as benign (axis Many national sports organizations now provide for such
deviation, atrial enlargement, and right ventricular testing of their elite competitors. At the same time, no
hypertrophy).34-36 These new criteria decreased the false- national sports organizationsdincluding those responsible for
positive rate from 17.3% to 4.5%.38-41 The 2010 ESC scholastic, college, and university sportsdmandate electro-
recommendations attempted to differentiate between electro- cardiographic screening of all competitors in their respective
cardiographic features suggestive of athletic training (type 1 programs. Major professional sport organizations in Canada
changes) from those possibly indicative of cardiac disease (type typically use a sophisticated systematic approach to the pre-
2 changes).42 Type 2 electrocardiographic patterns prompt participation evaluation of their athletes using teams of
referral for comprehensive evaluation before clearance to medical specialists and a battery of tests, including ECGs and
404 Canadian Journal of Cardiology
Volume 32 2016

echocardiograms. Junior hockey organizations vary in their all active youth, while recognizing the large numbers of young
approaches to the cardiovascular evaluation of their players. Canadians at risk of SCD who do not participate in sports.
It is useful to consider the implications of recommending a The prevalence of the diseases most likely to contribute to
12-lead ECG for young Canadians at the time of SCD in Canada, which is a multiethnic community, is not
commencement of signicant sports participation. There are known. In the interim, it seems appropriate for national sports
currently 750,000 Canadians engaged in school sports, organizations to implement programs of electrocardiographic
> 19,000 student athletes in Canadas 47 universities and 94 screening for elite competitors. Appropriate Canadian clinical
colleges, and > 500,000 participants in minor hockey. and public health organizations should be encouraged to
Countless numbers of young Canadians are also involved in continue to address the challenge of SCD among the young.
many other community sports and recreational programs The approach to, and target population for, cardiovascular risk
involving dance and other forms of vigorous activity. If elec- screening related to athletic participation is controversial, with
trocardiographic screening were to be recommended for every major geographic variations. Canadian-specic guidelines are
young Canadian involved in sports, signicant costs will be clearly needed.
incurred. If one obtained an ECG at the time of a young
athletes entry to high school, eg, at least 150,000 ECGs
would be obtained annually at a cost of more than $1.6 Disclosures
million. Notwithstanding the issues surrounding electrocar- The authors have no conicts of interest to disclose.
diographic interpretation, this would pose a logistic burden
for Canadas 1374 cardiologists. A considerable number of the References
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