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The Atlantic Rift: Guidelines for Athletic

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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
witnessed death of an apparently healthy individual occurring
within 1 hour of symptom onset or an unwitnessed death
occurring within 24 hours. Sudden cardiac death in athletes is
the leading cause of nontraumatic death, with a wide range of
incidence reported, from 1 in 3000 in some subpopulations to
1 in 1 million. Male persons, blacks, and basketball players
seem to be at a higher risk.1 Although rare, such deaths have a
devastating impact because athletes are perceived as epito-
mizing good health; the sudden unanticipated death of an
athlete precipitates questions and concerns often spurred by
extensive media coverage. These deaths are particularly tragic
because the majority result from inherited or congenital
cardiac diseases that are detectable and for which several
Received for publication December 11, 2015. Accepted February 15, 2016.
Corresponding author: Dr Paul Poirier, Faculty of Pharmacy, Laval
University, Institut Universitaire de Cardiologie et Pneumologie de Quebec,
2725 Chemin Ste-Foy, Quebec City, Quebec G1V 4G5, Canada. Tel.: 1-
418-656-4767; fax: 1-418-656-4581.
E-mail: paul.poirier@criucpq.ulaval.ca
See page 404 for disclosure information.
therapeutic strategies are available to minimize the risk of
death. The widespread publicity after such a death frequently
includes calls for more effective screening of athletes. In most
instances, however, there are no prodromal warning
symptoms, and SCD is the rst manifestation of disease in
young athletes,2 in contrast to middle-aged nonathletes in
whom warning symptoms (chest pain, dyspnea) frequently
occur before SCD.3 Of note, coronary disease events are
clustered around the nish line in mass-participation
events.4
Athletes, Recreational Sports Participants, or
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
high premium on excellence and achievement, and requires
some form of systematic (and usually intense) training. The
distinction between competitive athletes and those involved
in recreational sports lies in the ability and freedom of a
participant to judge when it is prudent to reduce or stop
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.
Guidelines for Athletic Screening
abnormal 12-lead ECG triggers further examinations, which are
expensive given the low diagnostic yield of most abnormal electro-
cardiographic patterns. Universal screening of young athletes poses
logistic and nancial challenges. There are currently no Canadian
guidelines regarding preparticipation screening of athletes. Screening
of athletes ignores the much larger group of young nonathletes who
participate in vigorous recreational activity and who collectively
represent a population in which a much larger number of SCDs can be
predicted to occur. While waiting for the best screening approach in
Canada, increased awareness of and access to automated external
debrillators, along with training in cardiopulmonary resuscitation, can
help reduce the number of SCDs. In some jurisdictions, electrocar-
diographic screening has been eschewed in favour of such an
approach. Specic physician training in the eld of sports cardiology
with availability of experts throughout Canada may be a useful start.
We provide suggestions and call for the development of Canadian
guidelines by appropriate organizations.
as individuals who are engaged in exercise training and
competition on a regular basis, exercising > 10 hours per
week. Elite athletes are a subgroup of this population. Dif-
ferences in characterizing the sporting population likely
contribute to the large variations in the reported incidence of
SCD in sports. Athletes have been found to have a higher risk
of SCD compared with nonathletes but not in all studies.6-8
Although deaths among young competitive athletes are higher
than in their sedentary counterparts, the overall denominator
for all exercising individuals dictates that exercise-related SCD
occurs much more frequently in recreational athletes.9
Causes of SCD in Athletes
The causes of SCD in athletes include structural,
arrhythmogenic, and acquired cardiac abnormalities (Table 1).
Some athletes experience syncope, chest pain, dyspnea
disproportionate to the physical exertion, seizure, and palpi-
tations before an event.2,10 In athletes  35 years, most events
result from atherosclerotic coronary artery disease,2,11 whereas
in those < 35 years, genetic and acquired cardiovascular
abnormalities, particularly cardiomyopathies, are more
commonly responsible.12,13 Screening for a family history of
premature (< 40 years) cardiovascular disease and SCD
should occur at a young age, because most SCDs in young
athletes are caused by inherited cardiac disease.2,14
Magnitude of the Problem
Sudden death in sports is rare, becoming increasingly
prevalent with age. A literature review reveals large variations
in the incidence of SCD among competitive and recreational
athletes. Reported prevalence rates for SCD during sports vary
and reect methods of data collection, athlete demographics,
and sporting discipline. Unfortunately, most reports regarding
SCD in athletes are derived from voluntary reporting or the
media. More detailed analysis of systematic registries such as
the Italian pathology registry in Veneto and the National
Collegiate Athletic Association Athletes registry reveals a
401
apparaissent chez les individus trs entrane s et qui se superposent
des resultats e
vocateurs dune pathologie. LGG 12 de rivations qui
est positif entrane dautres examens dispendieux e tant donne le faible
rendement diagnostique de la plupart des trace s anormaux dCG. Le
depistage universel des jeunes athltes pose des difculte s logistiques
et nancires. Il nexiste actuellement pas de lignes directrices cana-
diennes concernant le de pistage des athltes. Le de pistage des ath-
ltes ne tient pas compte du groupe plus nombreux de jeunes non-
athltes qui participent des activites re
cre
atives vigoureuses et qui
representent collectivement une population chez qui lon peut pre dire
la survenue dun nombre beaucoup plus grand de MSOC. Dans lat-
tente dune meilleure approche de de pistage au Canada, une sensi-
bilisation accrue et un accs aux debrillateurs externes automatiques
ainsi quune formation en RCR peuvent aider re duire le nombre de
MSOC. Certaines autorite s ont rejete
 le depistage par CG en faveur
dune telle approche. Une formation particulire des me decins dans le
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.
prevalence of 3-4 per million population.15,16 Insofar as the
debate for screening is concerned, the discussion needs to be
guided by the prevalence of young athletes harboring disease
rather than the prevalence of SCD. Several studies have
reported that 1 in 300 young athletes and nonathletes are
affected by inherited or cardiac diseases capable of causing an
exercise-related SCD. The considerably lower SCD rate
reects the fact that most diseases implicated in SCD in the
young have low event rates. The lowest rate of SCD among
young competitive athletes (0.46 per 100,000 athletes per
academic year) in high school grades 10-12 has been reported
in Minnesota.17
Table 1. Causes of sudden cardiac death in athletes
Inherited: structurally abnormal heart
Cardiomyopathies
Arrhythmogenic cardiomyopathy, right ventricular cardiomyopathy, or
dysplasia
Dilated cardiomyopathy
Hypertrophic cardiomyopathy, idiopathic left ventricular hypertrophy
Left ventricular noncompaction
Other
Coronary artery abnormalities
Valvular heart disease (bileaet mitral valve prolapse syndrome, bicuspid
aortic valve)
Aortopathies (eg, ascending aortic aneurysm, Marfan syndrome)
Inherited: structurally normal heart
Channelopathies
Brugada syndrome
Catecholaminergic polymorphic ventricular tachycardia
Idiopathic ventricular brillation
Long QT syndrome
Acquired: structurally abnormal heart
Ischemic heart disease
Myocarditis
Exercise-induced arrhythmogenic right ventricular cardiomyopathy
Acquired: structurally normal heart
Commotio cordis
Substance abuse
Environmental factors (eg, hypothermia or hyperthermia, electrolyte
disturbances)
402
Current Practice and Models (United States vs
Europe)
Although SCD during sports is rare, the societal impact of
such events, loss of decades of life-years, and the ability to
detect and manage the diseases implicated in SCD have
caused both American and European cardiac societies to
endorse preparticipation cardiovascular screening. Although
only 2 countries in the world (Italy and Israel) practice state-
sponsored screening, most elite sporting organizations in the
Western world recommend some form of screening of elite
competitors.18,19 Screening programs may involve all or a
combination of the following as preliminary investigations: a
health questionnaire inquiring about family and personal
history, physical examination, and a 12-lead ECG. Athletes
with abnormal results are subject to further examinations such
as echocardiography, stress testing, 24-hour Holter moni-
toring, and cardiac magnetic resonance imaging (MRI) to help
conrm or refute the presence of a potentially serious cardiac
abnormality. The American Heart Association (AHA) and the
European Society of Cardiology (ESC) both advocate
screening of elite athletes. However, they differ in that the
AHA recommends taking a thorough medical and family
history along with a physical examination, whereas the Eu-
ropean Society of Cardiology recommends the addition of a
12-lead ECG in the initial screening stages.20,21 A recent
AHA/American College of Cardiology (ACC) statement
reafrmed their position,22 noting that there is no compelling
reason to conne screening to young competitive athletes and
exclude nonathletes.23 The American model is cheap and
pragmatic but has poor sensitivity, with values as low as 6%.
Most athletes are asymptomatic before SCD, and most dis-
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,
family members may not have presented with a sentinel event.
In a seminal article, Maron et al.24 described the de-
mographics of 134 young athletes with SCD. Of them, 115
had been screened in accordance with the AHA recommen-
dations, and a correct diagnosis was identied in only 1
(0.9%).
Although controversy remains regarding the screening
ECG, all experts agree that the preparticipation screening
should include a cardiovascular-oriented history and physical
examination (Table 2).20 However, the AHA questionnaire
may yield a very high rate of positive responses.25 Approxi-
mately 25% of athletes screened with AHA personal and
family history elements may be referred for cardiovascular
evaluation so that approximately 2.5 million athletes would be
referred for further cardiovascular evaluation, in contrast to
the extremely low incidence of SCD in athletes. To our
knowledge, there has been no evaluation of the appropriate-
ness of the AHA-14 elements or the Stanford
questionnaire.25,26
Efcacy of the ECG
Addition of a 12-lead ECG to the prescreening process has
the potential for detecting athletes with electrophysiological
cardiac disease, such as the long QT, Brugada, and Wolff-
Parkinson-White syndromes. This may be particularly
Canadian Journal of Cardiology
Volume 32 2016
relevant because there is emerging evidence that most sport-
related SCDs result from sudden arrhythmic death syn-
drome, most commonly caused by ion channel diseases.12,18
Although cardiac imaging studies are the gold standard for
identifying cardiomyopathies, it is well recognized that at least
90% of patients with hypertrophic cardiomyopathy and 50%
of patients with arrhythmogenic right ventricular cardiomy-
opathy have an abnormal ECG. Cardiac screening studies
from Italy,18 the United Kingdom,27 the United States,28
Australia,29 and the Netherlands have shown that electrocar-
diography is superior to screening based solely on a health
questionnaire and physical examination for detecting athletes
with potentially serious cardiac disease.30 In the large 30-year-
old state-sponsored screening program in Italy, an ECG
detected > 80% of cases of hypertrophic cardiomyopathy.31
In a meta-analysis of 15 screening studies in > 45,000 ath-
letes, an ECG was 5 times more sensitive than a history and
10 times more sensitive than a physical examination and had a
higher positive likelihood ratio, a lower negative likelihood
ratio, and a lower false-positive rate.32 A total of 160 poten-
tially lethal cardiovascular conditions were detected (0.3%).
The authors suggested that a 12-lead ECG should be
considered best practice in screening athletes, whereas the use
of history and physical alone should be re-evaluated.32 One
must remember that the most frequent anomalies found in
this meta-analysis were Wolff-Parkinson-White syndrome
(42%), long QT syndrome (11%), and hypertrophic cardio-
myopathy (11%), which are unlikely to be found by history
and physical examination alone. The positive predictive value
of most apparently abnormal electrocardiographic patterns is
low (< 5%); however, deep T-wave inversion in the inferior
Table 2. The 14-element American Heart Association
recommendations for preparticipation cardiovascular screening of
competitive athletes
Medical history (parental verication is recommended for high school and
middle school athletes)
Chest pain/discomfort/tightness/pressure related to exertion
Unexplained syncope/near-syncope judged not to be of neurocardiogenic
(vasovagal) origin; of particular concern when occurring during or
after physical exertion
Excessive exertional and unexplained dyspnea/fatigue, associated with
exercise
Previous recognition of heart murmur
Elevated systemic blood pressure
Previous restriction from participation in sports
Previous testing for the heart ordered by a physician because of family
history
Premature death (sudden and unexpected or otherwise) before 50 y of age
attributable to heart disease in  1 relative
Disability because of heart disease in a close relative aged < 50 y
Hypertrophic or dilated cardiomyopathy, long-QT syndrome, or other ion
Physical examination
Heart murmur
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
tract obstruction
Femoral pulses to exclude aortic coarctation
Physical stigmas of Marfan syndrome
Brachial artery blood pressure (sitting position), preferably taken in both
arms
Poirier et al.
Guidelines for Athletic Screening
or lateral leads, or both, has a relatively high predictive value.
In a recent study of 155 athletes with deep T-wave inversion
predominantly in the inferior or lateral leads, or both, 41% of
athletes were diagnosed with cardiomyopathy after echocar-
diography and cardiac MRI testing. Other electrocardio-
graphic patterns highly suggestive of cardiac pathologic
conditions include ST-segment depression, pathologic Q
waves (Q/S > 0.25), left bundle branch block, and a corrected
QT interval  500 ms.33
The ECG is effective in detecting disease, but there is
debate regarding whether early diagnosis equates to a life
saved. Data from the mandatory Italian screening program
is the best evidence that screening may save lives. In a
prospective study using a pathology registry for SCD in the
Veneto region of Italy, the incidence of SCD among ath-
letes screened with electrocardiography fell from 3.6 per
100,000 to 0.4 per 100,000, representing a 90% reduction
in SCD.18 In contrast, a similar electrocardiography pro-
gram in Israel showed no difference in the prevalence of
SCD in athletes when comparing deaths rates 12 years
before screening vs 12 years after screening was imple-
mented.19 Determination of the numerator of SCD in the
study from Israel relied solely on reports in 2 newspapers;
therefore, it is likely that many deaths 12 years before
screening were not detected because media reporting during
that period was not as developed.
Concerns Relating to Electrocardiographic
Screening
Although the ECG is superior to screening with a health
questionnaire and physical examination alone, there are
several concerns relating to nationwide electrocardiographic
screening, including false-positive results, false-negative re-
sults, cost, and lack of infrastructure. In Europe, the cost of a
12-lead ECG is wV10 vs US$39-$47 in the United
States.21,34 The cost per life saved is estimated at $10.6-$14.4
million in the United States.34 Data from the Italian study
suggest that 33,000 athletes need to be screened to save 1 life
at a cost per life saved of $1,320,000.35 This money may be
better spent in secondary prevention of SCD in sports or
allocated to urgent widespread health care needs like the
increasing rates of obesity in the young.36
The athletes ECG reects increased vagal tone and
increased cardiac chamber size. Some athletes may show
profound repolarization changes that overlap with cardiomy-
opathy or ion channel diseases. Several publications provide
electrocardiographic criteria for evaluation of athletes. The
rst electrocardiographic criteria appeared in 1998 and were
adapted by the ESC in 2005.21 More recently, a multiple-
association working group produced the further rened
Seattle Criteria.37 Electrocardiographic patterns have now
been recognized that should be classied as benign (axis
deviation, atrial enlargement, and right ventricular
hypertrophy).34-36 These new criteria decreased the false-
positive rate from 17.3% to 4.5%.38-41 The 2010 ESC
recommendations attempted to differentiate between electro-
cardiographic features suggestive of athletic training (type 1
changes) from those possibly indicative of cardiac disease (type
2 changes).42 Type 2 electrocardiographic patterns prompt
referral for comprehensive evaluation before clearance to
403
compete. The initial estimated prevalence of type 2 changes in
athletes of 5% has proved to be an underestimate. The data
were derived from a nonselect cohort of white athletes and
have been associated with unacceptably high-false positive
rates (9%-22%) in elite endurance athletes; in athletes of
African origin, false-positive rates may exceed 40%. When
factors like age, sex, sporting discipline, body habitus, and
ethnicity are considered,43 most type 2 electrocardiographic
patterns do not lead to a clear diagnosis after investigation.
The ECG is thus a poor tool for discriminating between
cardiac physiology and pathology. More recent criteria have
accounted for black ethnicity and provided less conservative
limits for an abnormal QT interval, which has resulted in a
signicant drop in false-positive rates in white athletes to 3%-
5%, but there remains an issue with black athletes. Isolated T-
wave inversions in leads V1-V4 in black athletes should in
most cases not prompt further evaluation of asymptomatic
individuals.44-46 The cost of follow-up investigations to
conrm or refute the presence of a serious cardiac disorder and
the potential to unfairly disqualify athletes from participating
in sports are problematic. There are also concerns relating to
the consistency in the interpretation of preparticipation ECGs
among experienced physicians.47 Even among sports cardiol-
ogists, the variability in interpretation is as high as 20%.48
Electrocardiographic screening is also associated with false-
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,
a resting ECG will not reveal incomplete expressions of
cardiomyopathy or catecholaminergic polymorphic ventricular
tachycardia. In some jurisdictions, electrocardiographic
screening has not been advocated, but there has been an
increased emphasis on addressing preventable deaths in the
young by emphasizing debrillator placement and training in
cardiopulmonary resuscitation, suicide prevention, and stra-
tegies to reduce motor vehicle accidents.48
Fundamental to the preparticipation screening debate is
the balance between (1) lives saved; (2) number of athletes
tested; (3) associated psychological, ethical, and legal issues;
and (4) cost. There is a grey zone between pathologic
conditions and athletic physiological adaptations, producing a
real clinical challenge.20 Multiple issues were captured in a
recent AHA/ACC publication regarding the 12-lead ECG as
a screening test in healthy young individuals.26
Where Should Canada Stand?
There are currently no guidelines regarding pre-
participation electrocardiographic screening in Canada. In
2013, the Canadian Academy of Sport and Exercise Medicine
(CASEM) made a global recommendation to screen national-
level, high-performance athletes with electrocardiography.
Many national sports organizations now provide for such
testing of their elite competitors. At the same time, no
national sports organizationsdincluding those responsible for
scholastic, college, and university sportsdmandate electro-
cardiographic screening of all competitors in their respective
programs. Major professional sport organizations in Canada
typically use a sophisticated systematic approach to the pre-
participation evaluation of their athletes using teams of
medical specialists and a battery of tests, including ECGs and
404
echocardiograms. Junior hockey organizations vary in their
approaches to the cardiovascular evaluation of their players.
It is useful to consider the implications of recommending a
12-lead ECG for young Canadians at the time of
commencement of signicant sports participation. There are
currently 750,000 Canadians engaged in school sports,
> 19,000 student athletes in Canadas 47 universities and 94
colleges, and > 500,000 participants in minor hockey.
Countless numbers of young Canadians are also involved in
many other community sports and recreational programs
involving dance and other forms of vigorous activity. If elec-
trocardiographic screening were to be recommended for every
young Canadian involved in sports, signicant costs will be
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
million. Notwithstanding the issues surrounding electrocar-
diographic interpretation, this would pose a logistic burden
for Canadas 1374 cardiologists. A considerable number of the
ECGs will be problematic, prompting further testing. Sig-
nicant numbers of young athletes would be withdrawn from
activity pending further examinations. The Italian experience
suggests that 33,000 athletes would need to be screened to
save 1 life at a cost of $1.32 million. In addition, the vast
majority of young Canadians not participating in organized
sports are frequently involved in vigorous recreational activ-
ities that pose risk to those with rare underlying cardiac
abnormalities.
An appropriate mid-Atlantic approach to the current
dilemma may be to ensure that Canadas major sports
organizationsdincluding those responsible for college and
university sportsdmandate a preparticipation examination
including an ECG in all athletes participating in elite
competition or varsity teams. This screening would serve the
interests of sports organizations and their athletes. It is dif-
cult to conceive that universal screening of all young athletes
would be possible in Canada given the costs to apply a
screening strategy that is questionable from a public health
perspective. There is clearly an urgent need for detailed
guidelines on athletic screening from relevant organizations
such as the Canadian Cardiovascular Society and CASEM. To
further address the issue of SCD in Canada, the Cardiac
Arrest Survivors with Preserved Ejection Fraction Registry
(CASPER) could be of use. CASPER is a Canadian national
registry involving 14 adult and pediatric centres that examines
phenotype-genotype correlation and assesses test performance
in familial sudden death and unexplained cardiac arrest.49
This study enrolls patients with apparently unexplained
cardiac arrest and no evident cardiac disease to identify the
pathogenesis of SCD through systematic clinical testing. It
would provide a unique opportunity to assess SCD and its
underlying pathophysiology in Canada provided that the
information regarding exercise practices is captured in the
database.
Conclusions
Debate regarding the use of preparticipation testing con-
tinues. In Canada, there is a need to address this issue with
particular reference to the costs (of all kinds) and benets that
would surround the introduction of a generalized approach for
Canadian Journal of Cardiology
Volume 32 2016
all active youth, while recognizing the large numbers of young
Canadians at risk of SCD who do not participate in sports.
The prevalence of the diseases most likely to contribute to
SCD in Canada, which is a multiethnic community, is not
known. In the interim, it seems appropriate for national sports
organizations to implement programs of electrocardiographic
screening for elite competitors. Appropriate Canadian clinical
and public health organizations should be encouraged to
continue to address the challenge of SCD among the young.
The approach to, and target population for, cardiovascular risk
screening related to athletic participation is controversial, with
major geographic variations. Canadian-specic guidelines are
clearly needed.
Disclosures
The authors have no conicts of interest to disclose.
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