Papadakis, M., Sharma, S., Cox, S., Sheppard,
M.N., Panoulas, V.F. and Behr, E.R. "The magnitude of sudden cardiac death in the young: a death
certificate-based review in England and Wales." Europace 2009 Vol.11, No.10, p1353-1358 [Abstract]
When symptoms do present they include chest pain (exercise related chest pain is a red flag), syncope / passing out, palpitations, breathlessness (disproportionate to the amount of exercise) and dizziness. However, 80% of young sudden cardiac deaths will occur with no prior symptoms.
There are international recommendations that all young people should be tested prior to participation in organised sport. In Italy this has been law since 1982 that all young people have an ECG prior to participation in organised sport.
A specialist doctor attends CRY screening events. Specialism is essential to minimise false positives and false negatives (i.e. people being told they require more tests when they don’t really need them, people being reassured when there is actually something wrong).
10-20% of people tested by CRY will require an ECHO based on their ECG and/or their medical questionnaire. 4% of people will require further tests in a specialist centre – this will include Exercise tests, holter monitors, mri.
One in every 300 of the young people that CRY tests will be identified with a potentially life threatening condition.
1 in 100 will have a condition that is less serious but could cause problems in later life. Although screening will not identify all young people at risk, in Italy, where screening is mandatory for all young people engaged in organised sport, they have reduced the incidence of young sudden cardiac death by 89%.
Sport does not cause young sudden cardiac death, but it significantly increases a person’s risk if they have an underlying condition.
The incidence of sudden cardiac death in the young is
estimated to be 1 in 100,000 per life year. This number is
likely to be an underestimate in UK, due to several important reason.
There is lack of national systemic registry for sudden death in the
young, and most of the reports are derived from deaths of high profile
athletes, media reports etc. Also, not all the post mortem
examinations are carried out by expert pathologists with a special
interested in sudden death in the young. Hence, some of the
uncommon conditions are easily missed. Finally, some of the disorders
which affect channels in the heart (such as congenital Long QT
syndrome, Brugada syndrome, etc) have structurally normal hearts, and
hence cannot be diagnosed during autopsy.
It is estimated that approximately 80%
of all non-traumatic sudden deaths in young competitive athletes are
due to inherited / congenital structural or functional
cardiovascular abnormalities. Hypertrophic Cardiomyopathy (HCM) accounts for 40-50% of all such
deaths (Shama et al, Br J Sports
The majority of young sudden cardiac deaths are due to inherited forms of
heart muscle disorder and irregular heart beat. Hypertrophic
Cardiomyopathy is the most common of these conditions. Research
in the USA by Dr Barry Maron shows that 1 in 500 people have
Hypertrophic Cardiomyopathy (Maron, 1995, Circulation).
In the UK unexplained "sudden death" - where there is no
cause identified - is frequently classified as due to natural causes.
Experts believe that the majority of these deaths are due to Sudden
Death Syndrome or Sudden Cardiac Death. Until the law is changed and coroners have to refer
hearts on to specialists we cannot find the true statistics.
A national programme for systematic
pre-participation screening of all young competitive athletes has been in
place in Italy since 1971. Research there confirms that ECG testing
is the most cost effective way to test for cardiac abnormalities (Corrado
et al, 1998, New England Journal of Medicine). A competitive athlete was defined as a
participant in an organised sports programme requiring regular training
and competition - thus school children would be
included in the programme.
Corrado, D., Basso, C., Pavei, A., Michieli, P.,
Schiavon, M. and Thiene, G. "Trends in Sudden Cardiovascular Death in Young Competitive Athletes
After Implementation of a Preparticipation Screening Program." The Journal of the American Medical Association 2006 Vol.296, No.13,
Italian law requires every athlete to have an annual "Fitness
Certificate" before they are permitted to participate in any
event. Sports clubs and schools have to take responsibility for their
students' fitness to participate. One pathologist, Professor Gaetano Thiene,
resolutely collated all statistical data on sudden death in young
people in the Veneto region of Northern Italy, and as a result of his
research he discovered that cardiovascular screening was effective in
identifying individuals at risk. (Corrado
et al, 1998, New England Journal of Medicine).
Sudden death is defined as
unexpected death occurring as a result of natural causes in which loss of
all functions occurred instantaneously or within six hours of the onset of
symptoms or collapse. (Shama et al, Br J Sports Med).
Those sufferers with symptoms are considered to be the
"lucky" ones, as once cardiac conditions are recognised,
precautions can be taken, and much can be done to prevent
complications and sudden death.