Young people do not drop dead suddenly, for no reason.  Katy Edwards spoke to parents who are campaigning for better heart screening programmes to tackle cardiac risk in the young.

When 33-year-old semi-professional footballer Aaron Gardiner collapsed and died following a pre-season training run earlier this summer, an initial post-mortem examination proved inconclusive.  The Ipswich father of three and AFC Sudbury star was subsequently revealed to have died from natural causes brought on by Cardiac Arrhythmia (heart failure). 

His death came almost exactly a year after that of another footballer, 28-year-old Manchester City and Cameroon footballer Marc-Vivien Foe, who collapsed during an international match in Lyon.  Medical teams struggled to resuscitate him but he died later in hospital.  His death stunned the football world and the millions of people watching the match. 

Foe (on the right in the picture) was at the peak of his career and outwardly fit and healthy.  A first autopsy failed to confirm a cause of death.  But two weeks later, a second autopsy found that he had a heart condition known as Hypertrophic Cardiomyopathy (HCM), an incurable condition that causes excessive thickening of the heart muscle. 

Sudden, unexplained deaths in the young may be more common than we have previously been led to believe, according to the charity Cardiac Risk in the Young (CRY).  CRY believes as many as eight young people (under 35) succumb to Sudden Cardiac Death every week.  Often they are talented sportsmen and women in their prime – apparently fit and healthy – with everything to live for. 

As many cardiac irregularities are only detectable in the living, post-mortems may be inconclusive and sudden, unexplained deaths are commonly attributed to “natural causes”.  Such cases are all the more devastating to bereaved families, as they have nothing to blame. 

Kate and Robi Fox, from Woodridge, are only too familiar with the confusion that follows such a tragedy.  Their daughter Laura was just 13 years old when her life ended abruptly.  The family had just returned from holiday in France, in August 1998, when Laura was taken from them. 

Kate recalled: “We had been home a couple of days and the kids were at a loss as to what to do.  We were going to go and play tennis and Laura and Mark (her 19-year-old twin) went to get changed. 

“Mark found her in her room on the bed.” 

At the time, the family thought she was unconscious and immediately telephoned the ambulance but it was likely that Laura had already been dead for around 10 minutes.  They tried to resuscitate her until help arrived, but it was too late. 

Laura, a pupil at Farlingaye High School in Woodbridge, had been a fit, healthy and immensely sporty teenager who enjoyed running, triple jump and netball.  That summer, she had won the 100m sprints at her school sports day and was considering joining a running club in Ipswich. 

For such a vivacious girl to be taken so suddenly and apparently without cause left those who knew her reeling in disbelief.

“You can’t comprehend it, as you can imagine,” said Robi, Laura’s father. 

To make matters worse, a post-mortem examination was unable to establish the cause of his daughter’s death. 

“In cases where there are no suspicious circumstances, or it isn’t suicide, they don’t look much further,” he explained. 

It becomes ‘natural causes’ or they look for something else they could attribute it to, like asthma or epilepsy.”

Laura’s family found more answers through their involvement with the charity CRY (Cardiac Risk in the Young.)  At a time when nothing seemed to make any sense, CRY’s support was invaluable, Kate said, as it enabled them to speak to other parents who had been through similar experiences. 

Bill McKenna, professor of cardiac medicine at St George’s Hospital in London and world expert on Sudden Cardiac Death in the young, took up Laura’s case.  The professor, who is also a patron of CRY, believed Laura was suffering from Long QT Syndrome, a disorder of the heart’s electrical rhythm, which would not have been detected at a post-mortem. 

“When we were told that was probably what it was, it was a relief in a sense, because at least then we understand a bit more,” said Kate. 

As most of the cardiac conditions that cause sudden death in young people are inherited, Laura’s whole family were subsequently screened.  Robi was found to have the potential for developing Long QT Arrhythmia and is now being monitored. 

The cardiac diseases that can cause sudden unexpected death fall into two broad categories.  The first are those that go undiagnosed in life but are revealed when post-mortem examination is carried out.  The second includes all other sudden unexpected and unexplained deaths.  It is thought that most of these are caused by irregular heart rhythms, as in Laura’s case.  For these, no cause of death can be established during a post-mortem. 

Currently there is no nationally agreed term for a Sudden Adult Death that cannot be explained by a post-mortem; instead such deaths come under the umbrella term Sudden Death Syndrome. 

There was a similar difficulty with defining cot death.  Now that it has been officially labelled as Sudden Infant Death Syndrome, doctors are able to collect data, identify causes and take steps to prevent it. 

“The World Health Organisation now need to recognise Sudden Adult Death Syndrome and then these figures can be recorded,” Robi said. 

Fit and healthy, they go so suddenly

When Laura died, the Fox family had not heard of any similar cases but they have now come to realise Sudden Cardiac Death is not uncommon, especially among young athletes. 

The CRY website gives a long list of promising sportsmen and women who were cut down at their physical peak. 

Take, for example, Robert Hayley, a 17-year-old rower who died shortly after competing in a race with crewmate Sir Steven Redgrave, former Olympic champion and CRY patron. 

Or cyclist Adrian Hawkins, 22, who was short-listed for the Barcelona Olympics cycling squad but died two weeks later, immediately after winning a major race. 

Or Laura Moss, 13, from Weymouth, a junior swimmer on the elite Olympic Swim 2000 squad, who died warming up at a school swimming gala. 

Or Andrew Gard, a gifted hockey and tennis player from Frinton-on-Sea, who was struck down two days before his 18^th birthday, in 1997. 

This list goes on. 

Sport in itself does not lead to cardiac arrest but it can act as a trigger for a young person to die by suddenly exacerbating an undetected condition. 

CRY was established in 1995 by Alison Cox, wife of Marx Cox, a British tennis ace in the 1970’s, after their son Steven was found to be a potential sufferer of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), probably the second most common cause of unexpected sudden death in the young. 

Steven, who was on the verge of a glittering tennis career himself, was tested during a routine screening programme whilst at a college in America on a sports scholarship.  His parents believe that had he not gone to the US, he may not be alive today. 

CRY is campaigning for better screening for youngsters in this country to identify cardiac abnormalities before it is too late.  It believes the NHS should extend those programmes to all children in their mid-teens.  Laura’s parents are now active CRY members. 

"You just feel as though you want to prevent other people having to go through the same thing,” said Robi. 

“If people could be screened earlier, something could be done.  Those years under 35 are usually the most competitive years of your life when you push yourself the most.  If you have an underlying condition, that is when it could materialise into a major problem.  The Italians and the Americans screen all their elite athletes as a matter of course.  It is starting to happen in this country; the Lawn Tennis Association is screening all its youngsters and I think some cricket clubs are as well. 

“We would like to see all sports organisations giving youngsters that opportunity and raising awareness so parents could make a decision as to whether they want their children screened.  Obviously that brings its own problems as they will then find the consequences of those results could be life-changing (i.e. giving up sport.)

“We didn’t have that choice.  If we had, Laura might not have died.”

Where abnormalities are detected, depending on the nature of the problem, drugs may be used to regulate the heart, or the person may need an implantable device (Implantable Cardioverter Defibrillators) to automatically ‘shock’ the heart out of dangerous rhythms. 

One patient to have benefited from early detection is 14-year-old Laura John, from Southampton, who, together with her sister, was identified as having Long QT Syndrome in 1998.  Their mother fought for the girls to undergo the life-saving tests after their father died suddenly at the age of 32 from the same condition.  The sisters have now been fitted with life-saving cardiac implants.  Laura was given the prestigious task of tossing the coin for the men’s singles final at Wimbledon this year, as a representative for CRY. 

Thanks to CRY’s persistence, awareness is growing.  CRY has established a centre for sports cardiology at the British Olympic Medical Centre.  Six CRY clinics (including one at Colchester Hospital) also now offer either ECHO or ECG screening for people aged 14 – 35, or for athletes – an ECG test costs £35 and an ECG and ECHO, around £200. 

Following a Bill tabled in the House of Commons in March, a working group has been set up to advise on future policy on Arrhythmia and Sudden Cardiac Death in the young.  Its findings could lead to a new National Service Framework (NSF) chapter setting out the standards and models of care for such conditions. 

CRY heralded the move as a huge step towards greater recognition of the problem. 

Closer to home, Kate and Robi have worked hard to raise funds for CRY screening weekends in Ipswich and are hoping to hold another session later in the year.  They are inviting sports organisations to register an interest. 

“It’s also about bringing awareness to GP’s,” Kate said. 

“Heart problems are not the first thing you usually think of in young, fit teenagers.” 

Cardiac Risk in the Young

Sudden Death Syndrome (SDS) is an umbrella term used for the many different causes of cardiac arrest in young people.  Unexpected death in people under 35 is also sometimes referred to as SADS (Sudden Adult Death Syndrome). 

In the UK unexplained sudden death is frequently recorded as due to death from natural causes.  Until the law is changed and coroners have to refer hearts on to specialists we may not know the true figures.  Sudden cardiac death is defined as an event that is non-traumatic, non-violent, unexpected, and resulting from sudden cardiac arrest within six hours of previously witnessed normal health.  Two main conditions cause sudden cardiac death in young people: Firstly, the thickening or abnormal structure of the heart muscle (Hypertrophic Cardiomyopathy or HCM, and dilated cardiomyopathy, believed to affect around one in 500 young adults.) 

Secondly, irregularities of the electrical impulses that upset the natural rhythm of the heart.  Disorders of the electrical conduction system of the heart that can cause arrhythmias include Long-QT Syndrome or Wolff-Parkinson-White syndrome.  These disorders are thought to affect around 112,000 people. 

Once they have been detected, via an electrocardiogram (ECG) or echocardiogram (ECHO – an ultrasound of the heart), some of these disorders may be treated either with drugs or by an implantable cardioverter defibrillator (ICD) to automatically ‘shock’ the heart out of dangerous rhythms. 

Symptoms of cardiac disorders can include excessive breathlessness, chest pain, palpitations, fainting fits, blackouts and dizziness while exercising.  Where there is an underlying problem, loud noises or other physical or emotional “shocks” can lead to potentially life-threatening adrenalin surges. 

CRY offers support to those who have suffered a loss, through a network of affected families and counselling.  It also promotes heart screening, ECG testing, contributes to medical research and donates medical equipment to doctors' surgeries and hospitals.