The sudden death of footballer Marc-Vivien
Foé last week was no freak one-off. More than 3,000 apparently young
healthy adults die unexpectedly every year. Should we be worried, asks
Sophie Petit-Zeman
When former Wales football manager Terry Yorath saw 28-year-old Cameroon
soccer star Marc-Vivien Foé crumple to the ground during last Thursday's
match against Colombia, it brought back especially painful memories.
Eleven years ago, Yorath's 15-year-old son, Daniel, who also had a
promising soccer career ahead of him, died while kicking a football around
the back garden with his father. "The way he collapsed was exactly
the same way as Daniel collapsed," says Yorath, who is now calling
for more stringent and regular tests in order to safeguard players'
health.
While experts try to establish exactly why
Foé died – despite being at the peak of fitness and apparently unaware of
any warning symptoms – his death has highlighted the small but worrying
number of cases of young, otherwise healthy adults, some of them athletes,
though not all, who die abruptly and unexpectedly every year. They don't
display symptoms, they seem the least likely victims, but a hidden
timebomb inside their bodies suddenly blows and there is little or no
chance to save them.
According to the British Heart Foundation
(BHF) about 3,500 apparently healthy adults die suddenly every year in
England. Postmortems can identify the cause of death in most of these,
with inherited disorders of heart structure most often to blame. Indeed,
Daniel was found to have such a condition, called Hypertrophic Cardiomyopathy
(HCM). According to Professor William McKenna, president of
the Cardiomyopathy Association and professor of cardiology at University
College, London, HCM affects as many as one in 500 people, but is severe
enough to put only a small number of these at risk of sudden death. In HCM,
the left ventricle, one of the heart's main pumping chambers, and the
septum, which divides the heart down the middle, become thickened. This
leads to a fast, irregular heartbeat and ultimately reduced output of
blood from the heart.
While structural heart abnormalities are
far and away the most common cause of sudden death in adults, in about 150
cases annually no cause is found at postmortem. In these cases, if the
problem originates in the heart, it is due to electrical abnormalities.
The irony of this is that such problems are only detectable when people
are alive, so without investigation during life it is never known that
they were to blame.
Dr Tim Bowker, BHF associate medical
director, wants all such deaths to be recorded and classified in the same
way as cot death , which became formalised as sudden infant death
syndrome. "Following the official classification of cot deaths as
sudden infant death syndrome, deaths fell by 70% over 10 years,"
says Bowker. "Not until it is accepted practice to identify all these
unexplained deaths and to label them as such, will it become possible to
study them systematically, identify their causes and find ways of
preventing them from occurring – and the name we propose is 'sudden adult
death syndrome' or 'SADS'."
The kinds of heart abnormalities that might
cause sudden death include conditions such as long QT syndrome and
idiopathic ventricular fibrillation, which could be identified in
apparently healthy people by simple tests such as electrocardiograms
(ECG), at rest and during exercise. Alison Cox, founder and chief
executive of Cardiac Risk in the Young (CRY), is a former professional
tennis player who is calling for such tests to be offered to all young
people between puberty and 35 years of age. "About eight healthy
young people die suddenly each week, and Cry's dream is that all
school-leavers have an ECG to identify those at risk."
While others feel that such calls are
alarmist, and that focusing screening on people with family histories of
heart disease is more appropriate, Cry's mobile testing facilities have
proved popular since they were introduced in 1993. As Cox explains:
"Our screening teams go out to sports clubs, but also general
organisations and even into communities after a young adult dies
suddenly." It costs about £200 for a comprehensive screening per
person, but the Cry service also offers a basic ECG (which Cox says is
enough to suggest whether further screening is needed) and follow-up
ultrasound if this is necessary for £35 a head.
Cry does not offer genetic screening as
part of its package but this may become increasingly important as more
genes associated with heart disease are identified.
McKenna is attempting to implement genetic
diagnosis into clinical practice for heart conditions linked to sudden
death through the work of the London Ideas Genetics Knowledge Park based
at London's Institute of Child Health. "HCM is an obvious candidate
for genetic testing, as mutations in about 10 genes have now been shown to
cause it. Screening individuals from at-risk families allows us to
identify the minority of those at risk from sudden death and to offer them
life-saving care such as fitting a specialist pacemaker which can
recognise a potentially lethal heart rhythm and deliver a small shock to
restore it to normal. Testing also helps us to understand more about the
links between the condition and the genes which underlie it."
There is also interest in the possibility
that over-the-counter remedies for common complaints such as hay fever or
indigestion could trigger potentially fatal heart rhythms in at-risk
adults. Fears about these links have already led to the withdrawal of
leading remedies such as Triludan (an antihistamine prescribed for hay
fever) and Prepulsid (often taken for acid indigestion). However, some
drugs, such as treatments for malaria or antibiotics for people allergic
to penicillin, cannot be banned from widespread general use and it is
therefore vital that at-risk individuals can be identified early so that
GPs can consider alternative treatments.
A new research study, based at St George's
Hospital Medical School, London, and the Drug Safety Research Unit (DSRU)
in Southampton, is now asking all consultant physicians and GPs throughout
England to help them to identify patients who develop heart rhythm
problems after taking medicines which are known to have this serious side
effect.
Professor Saad Shakir of the DSRU, says:
"This project is vitally important, not only because it has the
potential to save lives in people who may have a yet undetected risk
factor, but also to avert unnecessary panic about those drugs that are
largely safe and beneficial to the majority of the population.
"We do not want to see drugs withdrawn
from the market, especially if there is not a viable alternative for
patients. We must remember it's not these remedies per se that pose a
danger, but simply a small minority of people who cannot tolerate their
effects. We therefore hope this research will enable us to identify those
people and ensure they are protected from the risk."
If the cause of sudden death cannot be
found in the heart or related to drugs (legal or illicit), the next place
to look is in the brain. The most common neurological cause of sudden
death in healthy young people is bleeding over the surface of the brain or
within it. This may follow rupture of a weakened blood vessel or very
occasionally be the result of some kind of malformation within the brain's
blood vessels. Some young people also have more traditional strokes, in
which a blood vessel is blocked and the blood and oxygen to the brain cut
off. All the usual risk factors for stroke, such as smoking, high blood
pressure, high cholesterol and so on, can be to blame, which suggests that
in a small number of cases there are things that could have been done to
reduce risk.
Sudden death in seemingly well, young
adults like Marc-Vivien Foé is always difficult for those left behind. We
are edging closer to understanding causes, risk factors and preventive
strategies in many cases. But there will still remain a few utterly
baffling cases, for which no explanation can be found and little comfort
derived.
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