Youth struck down in its prime

The recent passing of a national serviceman put the spotlight on sudden cardiac death, the leading cause of fatalities among young and teenage athletes in the US. A lack of obvious symptoms makes it particularly difficult to treat, Hareth Al Bustani and Mitya Underwood write

Distressed players look on as medical staff attend to Fabrice Muamba who collapsed during an FA Cup quarter-final match in 2012. His life was saved due to good fortune and good planning. Suzanne PlunkShow more

Barely two minutes before half–time in the English FA Cup quarter–final tie between Bolton Wanderers and Tottenham Hotspur, the Bolton midfielder Fabrice Muamba, in the words of one spectator, “fell like a tree trunk”.

From the moment he hit the pitch on that Saturday afternoon two years ago, Muamba’s chances of survival were slim.

That he lived was due to good fortune and good planning. A team of medical staff, equipped with a defibrillator, was on duty in the stadium, while watching from stands was a cardiologist who persuaded the ambulance crew to divert to a specialist heart unit rather than the local hospital.

Those circumstances raised Muamba’s chances of survival from barely 10 per cent to 64 per cent. And survive he did, although he retired as a professional footballer on medical grounds just a few months later.

Many others are not so lucky. This week came the news that a young Emirati undergoing training for national service had collapsed and died, apparently without warning.

According to the uncle of Mohammed Khalid Al Risi, his 18-year-old nephew’s tragic death was not without precedent in the family from Al Ain. According to the uncle, at least two of Mohammed’s uncles had died from sudden heart attacks, including one as recently as this summer.

Another had died while serving in the military and was just three days from completing his training when “he fell right in the middle of the square, just like Mohammed”, according to Ahmed Saeed.

Such deaths hitting the young and apparently healthy are particularly hard on the families they affect. Yet while they are thankfully rare, they are far from unknown. Often the trigger is physical exertion.

In the United States, sudden cardiac arrest is the leading cause of death among young and teenage athletes.

Ordinarily, a healthy heart will pump blood throughout the body and lungs uninterrupted. This is powered by a cyclic flow of electricity, which passes through the atria, the top chambers of the heart, triggering a contraction that pumps blood into the ventricles, the bottom chambers.

The electric impulse then goes through the AV node, an “electrical relay station”, which slows the signal and allows it to continue to the ventricles; pumping blood through the right ventricle to the lungs and through the left ventricle to the rest of the body.

When this electricity flow is disrupted it can damage the heart’s ability to continue pumping and, hence, the heart rate. This is known as arrhythmia, which affects people who suffer cardiac conditions that run in the family. Because of a lack of obvious symptoms, these are often undiagnosed until after the person has died.

Such incidents are referred to as sudden cardiac deaths (SCD). For one in 20 cases of SCD, doctors are unable to determine any clear cause of death – these are known as sudden arrhythmic death syndrome (Sads).

Dr Jairam K Aithal, a cardiovascular disease specialist at Burjeel Hospital, says the issue is compounded, not just in the UAE, but in all developing countries, by a lack of a centralised medical database. Having one, he says, would reduce the risk of complications, the time taken to diagnose problems, and would facilitate faster exchange of crucial patient information.

Sads is caused by ventricular arrhythmia – a cardiac arrest triggered by a disturbance to the heart’s rhythm. Cardiac arrest can arise when the heart has virtually no blood pressure, owing to a highly erratic heartbeat.

Four in every 10 potentially fatal arrhythmias are caused by rare genetic conditions that disturb the electrical flow of the heart. They are usually inherited and caused by genetic mutations. They cannot be discovered once a person has died, and often remain elusive while they are alive.

When the heart’s electric flow becomes abnormal, the affected person becomes vulnerable to arrhythmia, which can lead to blackouts, cardiac arrest and even sudden death.

“If an arrhythmia lasts for 30 seconds or more, it could be fatal,” Dr Aithal says.

Although there are many variations, the most common genetic condition is Long QT syndrome (LQTS), which occurs in one in 2,000 people. In 70 per cent of sufferers, the cause can be identified. While there are no symptoms for Long QT syndrome, a rare form called Andersen’s Syndrome can cause muscle weakness or abnormalities in the toes, fingers, chin or skull.

Dr Wael Al Mahmeed, board member of the Emirates Cardiac Society, has been dealing with heart patients in the UAE for almost 20 years after working in Canada and the UK.

He says rates of sudden cardiac deaths here are probably on a par with international averages but research currently under way would give a better picture. Dr Al Mahmeed is also leading a study into Brugada syndrome, a condition sometimes linked to chemical imbalances or an inherited gene, that he says his department treats a lot.

People with this syndrome have an increased risk of arrhythmia, which can cause complications such as fainting or a sudden cardiac arrest. It is more common in males and in those with a family history, but is manageable if diagnosed early.

“We have seen a lot of cases here and we are making a registry, then next we are going to start doing genetic testing on those people and their relatives.”

The samples, which have to be sent abroad for testing, should show the doctors whether there is a higher incidence of the syndrome here than elsewhere.

Half of the families who lose a member to Sads share inherited heart disease. While the other half do not show signs, this does not mean they are safe.

The best way to check is to first investigate what might have triggered the family member’s death. Following this, family members should undergo examination for inherited structural heart diseases, which cause between 10 and 20 per cent of Sads deaths.

LQTS can be diagnosed by scanning the heart’s electrical activity. During an electrocardiogram (ECG), sensors are taped to the patient’s legs, arms and chest, to take heart readings. However, this does not always identify carriers.

There are many different tests that can be taken, including an echocardiogram – which scans the heart structure with ultrasound waves.

Dr Aithal says if everyone was thoroughly tested, 80 or 90 per cent of people with arrhythmic conditions would be identified.

Dr Al Mahmeed says there are three simple things the country can do to reduce sudden cardiac deaths.

The first is vocational training for people to learn how to perform basic life support. “Nothing beats basic life support. When somebody crashes and a passerby does basic life support, it can really help.

“This is the most important thing. We should say to our community ‘go do a basic life-support course’. It’s not rocket science, and it can make a very big difference.”

The likelihood of sudden cardiac death can be raised by exercise, although many deaths occur during the victim’s sleep. Those who know they are susceptible to this can take home defibrillators, which are used to shock the heart back into a normal rhythm following cardiac arrest.

Dr Al Mahmeed says the second important thing to reduce the numbers of sudden cardiac deaths is improving access to these defibrillators.

Automated external defibrillators (AEDs) are small and designed to be used by non-medical people. In some countries, including parts of the US, hotels, schools, swimming pools and police stations are required by law to have an AED on the premises.

There are also mobile phone applications that can alert the user to the nearest AED. The AED Locations app in New Zealand identifies the locations of AEDs using data submitted by the machine owner.

The UK’s South Central Ambulance Service launched its own iPhone app that locates more than 600 defibrillators in four counties. As well as the address of the machine, the app also tells the user where to find it in the building.

Statistics show that the chances of survival for someone who has a sudden cardiac event increases from one in 10 to four in 10 if someone uses an AED before ambulance crews arrive.

Last year Sheikh Saif bin Zayed, the Deputy Prime Minister and Minister of Interior, ordered the public to be trained to use AED devices after a decision to install them in public areas across the country.

“Having the availability of defibrillators in open areas such as football pitches, clubs and airports would certainly help reduce sudden cardiac deaths,” Dr Al Mahmeed says. “This is happening. It’s not very cost effective and you can’t put them everywhere, but it’s better than nothing.”

However, he adds, the country also needs to implement better screening, especially of relatives of people who have died in those circumstances.

“If they think it’s a sudden death, relatives should be screened.”