AMANDA
BELGER was a healthy and fit 21-year-old mother of two young children when, a
year ago, she collapsed and died. There were, apparently, no warning symptoms.
Amanda's name became added to the list of those who have died from what has
become known as "sudden death syndrome" – a
list that includes four to six under the age of 35 a week.
Sandra
Armstrong, Amanda's mother, says: "The suddenness of the death was shocking
and bewildering."
One initial
suggestion was that Amanda had undetected epilepsy and her death was due to a
swift and fatal seizure. Sandra and her second husband, Godfrey Armstrong, a
musician, thought that explanation unlikely.
Last November,
six months after Amanda's death, a coroner concluded that she had had a sudden
and fatal cardiac arrhythmia caused by a disruption in the heart's wiring. The
specific abnormality is known as "long QT syndrome" and is inherited.
Subsequent tests
this year by Professor William McKenna, a cardiologist at St George's Hospital,
south London, and a leading researcher in sudden death syndrome, suggest that
Amanda's children – Liam, three, and Ella, now 15 months – have signs of the
same defect. The peak age for such deaths is between 12 and 35.
The children
will now be assessed regularly and, when old enough, may be treated with drugs
such as beta-blockers or may be fitted with a defibrillator, the size of a
matchbox, which can be implanted under the skin to kickstart the heart at the
first sign of trouble.
In the past,
such cruelly sudden deaths in young people were accepted as rare and
inexplicable acts of fate. Now, thanks largely to the charity Cardiac Risk in
the Young (CRY), we know that such deaths are more common than once thought. The
charity, supported by Prof McKenna and by sportsmen including Ian Botham and Sir
Stephen Redgrave, campaigns for more awareness among the public and GPs of the
risks and potential symptoms.
The charity
advocates a national screening programme for heart abnormalities in young people
and research into their prevalence and genetic histories. Despite the growing
awareness, many of the 200 or more unexplained sudden deaths a year are recorded
by coroners as due to natural causes. CRY says: "Until coroners are
required to refer the hearts in such cases to specialists, we will never know
the true figures."
Sudden death
syndrome is the umbrella term used for about 10 cardiac conditions affecting
heart muscles and the electrical activity of the heart. Some are less common
than others. Many who die suddenly are physically very active; some are
professional athletes or talented sporting teenagers. A few years ago, the death
of Daniel Yorath, 15, an aspiring footballer who had just signed for Leeds,
captured the headlines when he died playing in the garden.
Sport itself
does not lead to heart trouble, says Alison Cox, who founded CRY with her
husband, Mark Cox, the leading British tennis player in the Seventies. "But
pushing the body to physical extremes could exacerbate an undetected
abnormality." One specific defect known as "hypertrophic
cardiomyopathy" is the most common form and is said to account for about
half the sudden deaths in athletes.
When the Coxes'
son Steven, an outstanding junior tennis player, went on a sports scholarship to
an American college they learnt – just in time – about the risk. A few years
earlier, a student at the college died suddenly and, fearing litigation, the
college introduced screening for all young people on intensive sports programmes.
Steven was found to have early signs of a potentially dangerous abnormality. Now
27, he has given up his tennis career.
Dr Sanjay
Sharma, a consultant cardiologist at Lewisham University Hospital, explains that
in some young athletes, intensive training is associated with structural changes
in the heart that may simulate a suspicious heart problem.
He says:
"Giving reassurance that there is no risk in such cases is important, but
it should be done only by a cardiologist experienced in assessing young
athletes." Already, sports clubs are more alert to the legal and medical
risks, and many insist on heart tests for young athletes.
Sandra Armstrong
wished she had known of the long QT syndrome. "My daughter's death was
waiting to happen. There have been at least two unexplained sudden deaths in
Amanda's father's family." She says subsequent tests show that several
older members have signs of the syndrome, though they remain healthy.
Detection of
potential risk through an electrocardiogram, a recording of the heart's
electrical activity, is painless and relatively simple. And, at £35, it is
affordable, says CRY, which would like to see such testing introduced more
widely on the NHS.
Doctors look at
the ECG recording for unusual warning "blips" and if necessary carry
out further tests. These include an echocardiogram, an ultrasound check on the
movement of heart valves.
Sudden deaths
are shattering. The Armstrongs are now bringing up Amanda's children. Nothing,
says Sandra, can bring back her "lovely, good-looking young daughter",
but she is determined to speak up for others who may be at risk. "We have
to detect these abnormalities. Even if children are not tested, there are
symptoms such as fainting attacks, dizziness or palpitations that could herald
trouble. We need sharper focus by GPs on symptoms they often dismiss as
transient, and advice on how to avoid trouble if you are at risk.
"Sudden
loud noise is known to be a possible trigger of sudden death in those with long
QT syndrome. It is possible that a door blown shut downstairs might have
accounted for Amanda's death. Most people I know had never heard of these
disorders until Amanda died."
Spotting
heart defects early
This month, the
hearts of up to 1,000 students, aged 14 to 16, in state schools in the Western
Isles, are to be screened. The aim is to discover more about the prevalence and
potential risk of unsuspected heart defects.
Ethical approval
for giving the children ECG tests has been given, and soon parents will receive
letters explaining the research in detail and asking for their consent and
cooperation. The research is expected to take several years, with advice and
treatment for children considered at risk. CRY is supporting the study, which
will also consider the psychological impact of ECG testing.
The study, led
by Dr Sharma, sets a precedent. So far, screening in schools has only happened
at independent schools. CRY recommends that all young elite athletes should
consider screening. In some countries, such as Italy, it is mandatory for those
participating in sport. ECG tests for athletes are available at the CRY Centre
of Sports Physiology at the British Olympic Medical Centre in north London.
Experience with elite athletes, says Dr Sharma, suggests that about four per
cent of those tested need further investigation.
What is certain,
says Dr Sharma, is that the families of anyone who has died of sudden death
syndrome should have an ECG check. "We can classify the risk, treat if
necessary and advise on potential symptoms and how to stay out of trouble."
Symptoms include
excessive breathlessness, chest pain, palpitations, fainting fits, blackouts and
dizziness while exercising. Those with a problem are usually advised to avoid
situations that might lead to sudden and potentially life-threatening
"adrenalin surges".
Sudden deaths
have been reported following a swimmer jumping into cold water or sudden loud
noises, such as an alarm clock or doctor's bleep – this may have triggered the
death of one young doctor who apparently died suddenly in his sleep.
· CRY: 01737 363222; http://www.c-r-y.org.uk
