Cell Tissue Res (2012) 348:315318

DOI 10.1007/s00441-012-1398-4

REVIEW

Why and how to support screening strategies to prevent

sudden death in athletes
Gaetano Thiene & Domenico Corrado & Ilaria Rigato &
Cristina Basso

Received: 2 March 2012 / Accepted: 5 March 2012 / Published online: 29 March 2012
# Springer-Verlag 2012

Abstract Sudden death in athletes occurs because of the

existence of hidden cardiovascular disorders which, during
effort, may jeopardize the electrical stability of the heart,
triggering ventricular tachycardia and/or fibrillation. Apart
from rare conditions of ion channel diseases in the setting of
a structurally normal heart, in which the disorder may be
easily diagnosed on basal or stress test ECG, cardiac abnormalities at risk of causing sudden death may affect the aorta
(Marfan syndrome), the coronary arteries (congenital coronary artery anomalies, premature coronary atherosclerosis),
the myocardium (hypertrophic and arrhythmogenic cardiomyopathy), the valves (bicuspid aortic valve, mitral valve
prolapse) and the conduction system (pre-excitation syndromes). These structural heart disorders may be detected
by ECG and/or echo. The employment of these tools at preparticipation screening can help to identify concealed
anomalies, which may play a major role in early diagnosis,
risk stratification, and prevention of sudden death.
Keywords Athlete . Sudden death . Pathology . Screening .
Prevention

Young athletes death has raised a debate in the U.S. and

critics say that a wider screening including ECG could
detect several hidden heart defects and save lives.
There is a rationale for supporting such a screening
strategy. The following statement may be convincing.
1) Sport activity is healthy and should be recommended,
particularly in the young. However, there is a paradox
G. Thiene (*) : D. Corrado : I. Rigato : C. Basso
University of Padua Medical School,
Padua, Italy
e-mail: gaetano.thiene@unipd.it

in the exercise that is like a two-edged sword: it can

simultaneously offer protection for the risk of sudden
death in those who are regularly engaged and can
increase a short-term risk of sudden death due to
underlying heart disease.
2) Although rare, the risk of sudden death does exist
during sport activity (Corrado et al. 1990). The recent
report in the media of the video-recorded collapse and
death of a first-ranking athlete like Puerta, soccer
player in Sevilla, who was found at postmortem to
be affected by arrhythmogenic cardiomyopathy, represented a dramatic event. In the Veneto Region Prospective Research Project of sudden death in the
young, the incidence of the phenomenon in athletes
was calculated as 2.3/100,000/year, nearly 3-fold the
incidence of 0.9/100,000/year in non-athletes (Corrado
et al. 2003).
3) The higher incidence of sudden death in athletes vs
non-athletes gives evidence that effort may be the
trigger for life-threatening arrhythmias. This does not
mean that per se sudden death is due to effort; on the
contrary, it is related to the existence of concealed
cardiovascular abnormalities, either structural or functional, which act as predisposing factors to cardiac
arrest. Both autonomic imbalance and vigorous cardiovascular performance act as triggers during exercise.
The concealed defect affects the major components of
the heart: aorta, coronary arteries, myocardium, valves,
conduction system, ion channels (Corrado et al. 2005a).
4) The paradox is that a heart with subtle morbid entities
can exhibit optimal, even extreme myocardial contraction activity and cardiac output with excellent physical
performance. Conditions with cardiac failure or overt
ischemic heart disease (i.e., dilated cardiomyopathy,
obstructive coronary artery disease with previous

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myocardial infection) present with dyspnea, fatigue, or

angina in such a way as to limit exercise. It is the
abrupt onset of electrical instability with ventricular
fibrillation (heart delirium), not preceded by any
previous symptoms, which is life-threatening (Thiene
et al. 2010).
5) In the majority of cases these abnormalities are suspected or even diagnosed at basal 12-lead ECG (see
cardiomyopathy, ion channel disease, av block, preexcitation syndrome, etc.). The ECG is the easy way to
identify subjects at risk and may be considered a lifesaving tool (Corrado et al. 2008). Of course, there may
be alarming symptoms (syncope, palpitations, dizziness)
that should not be overlooked. This is the reason why

Fig. 1 International Society of Sports Cardiology flowchart

Cell Tissue Res (2012) 348:315318

an ECG should be included in routine pre-participation

screening of young athletes for eligibility or disqualification. Among 33,735 athletes examined in the Veneto
Region, according to the Italian screening program, 22
(0.07%) turned out to be affected by hypertrophic cardiomyopathy: 18 (82%) had abnormal ECG, whereas
only five (23%) had a positive family history or cardiac
murmur at the physical examination, with nearly
60% less sensibility (Corrado et al. 1998). The superiority of the Italian program (including ECG)
(Decree of the Italian Ministry of Health 1982) vs
the American program (without ECG) (Maron et al.
2007) is clear-cut. In arrhythmogenic cardiomyopathy, ECG abnormalities may even be superior to

Cell Tissue Res (2012) 348:315318

imaging techniques in detecting minor forms of the

disease with discrete fibro-fatty infiltration of the free
wall, in the absence of dyskinesia/aneurysms and
with preserved ejection fraction of the right ventricle
(Pelliccia et al. 2008).
6) Implementation in Italy of a preparticipation screening
program for sport eligibility since 1982 (Decree of
the Italian Ministry of Health 1982), which includes
ECG, resulted in 90% decrease of sudden death in
athletes, aged 2035 years, in the Veneto Region in
the time interval 19802004 (Corrado et al. 2006).
This was mostly due to the ECG detection of cardiomyopathies thanks to dissemination of diagnostic
criteria and awareness of the existence of these harmful morbid entities by sport physicians and cardiologists. Sudden death due to cardiomyopathy dropped
from 1.5/100,000/year in the pre-screening era to 0.15/
100,000/year of the late screening period (Corrado et
al. 2006). Nowadays, dangerous morbid entities such
as arrhythmogenic and hypertrophic cardiomyopathy
should no longer escape detection during the visit at
pre-participation screening. It is time to turn the page
in the approach to pre-participation screening for competitive sport activity by introducing not only physical
but also instrumental examination worldwide, with

Fig. 2 Different approaches to

prevention

317

ECG playing a pivotal role. Screening young athletes

with 12-lead ECG in addition to cardiovascular family
history and physical examination has been proven to be
cost-effective (Corrado & McKenna 2007).
7) There are potentially dangerous abnormalities such as
valve disease (mitral valve prolapse, bicuspid aortic
valve) that may be ECG-silent, thus escaping detection if only ECG is employed. In this setting, cardiac
murmur due to mild regurgitation should raise suspicion, justifying the recommendation of echocardiography as a diagnostic tool. Half of the young people
affected by bicuspid aortic valve present increased
diameter of the aortic root, even in the absence of
any murmur. Whether 2D echocardiography should
be used as a routine tool for screening, in addition to
ECG, should be taken into serious account, considering that most of those at risk present gross structural
deformities of the heart.
8) The existence of a link between ischemic heart disease
and lifestyle risk factors (smoke, obesity, drug abuse
like cocaine) should suggest blood lipid assessment and
in selected cases even CT coronary angiography as complementary investigations. Bear in mind that exercise
adds a 2.6-fold risk of sudden death to young people
affected by concealed atherosclerotic coronary artery

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disease and a 79.0-fold risk if affected by congenital

coronary artery anomalies (Corrado et al. 2003). The
sensitivity of ECG, both basal and stress test, in discovering concealed coronary artery disease is poor,
both for acquired (coronary atherosclerosis) and congenital anomalies. This represents a major limitation
and indeed sudden deaths still occurring in the athletes
of our Region are mostly due to undetected coronary
artery disease.
9) Up to 3040% of sudden cardiac death in athletes are
ascribable to heredo-familiar disorders, with or without structural abnormalities. Molecular mutation analysis as well as cardiological and genetic screening in
first-degree family members should be carried out to
reveal healthy carriers at potential risk and may be
life-saving (Basso et al. 2010).
10) Sports pre-participation screening, including genetic
screening when deemed necessary, aims not only
to detect and disqualify athletes at risk but also to
reassure the vast majority of healthy, unaffected people, who are then allowed to continue safely in the
pleasure of sports activity or profession (Corrado et al.
2005b).
A flow chart has been put forward by the International
Society of Sports Cardiology adopting the Italian protocol
of Cardiovascular Preparticipation Screening (Corrado et al.
2010) (Fig. 1).
Of course, physical examination, personal and family
history and 12-lead ECG represent a first-level investigation
to raise the suspicion of an underlying disorder. If positive, a
cascade of investigations then begins: second-level with
non-invasive tools (stress test, signal average ECG, echo,
cardiac magnetic resonance, coronary angio CT, genetic
analysis) and third-level with invasive tools (coronary
angiography, electrophysiological study, electroanatomic
mapping, endomyocardial biopsy), aimed at achieving a
definitive diagnosis.
The prevention of sudden death in the young and
athletes is nowadays feasible and can be accomplished
in different ways (Fig. 2): sport disqualification that acts
in removing exercise as a trigger, drug therapy or ablation
on arrhythmic mechanism and implantation of defibrillator
on cardiac arrest.
All of these are palliative interventions, which do not
influence disease prevention and cure. This represents a
major challenge for basic science and translational cardiovascular medicine.

Cell Tissue Res (2012) 348:315318

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