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 assudden 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.
