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Dr Amanda Varnava comments on SADS for The Times

July 23, 2015

Why do good men still die young?

David Frost's son, Miles, has collapsed and died during a run. Are such deaths preventable?

On a Saturday afternoon in May 2014 Andrew Dickinson, head of history at Easthampstead Park community school in Bracknell, was at home, contemplating taking his red pen to his pupils' exercise books. "He was going to do his marking," recalls his wife, Carys, "but it was a lovely sunny day so he went for a run first."
A fit 32-year-old, who ran half-marathons regularly and had played hockey at university, Dickinson had never had any indication of heart problems. However, on returning from his jog he collapsed outside his house in Farnborough, where he was found by neighbours. An ambulance took 22 minutes to arrive. He was resuscitated eventually, but died six days later in hospital. His son, Henry, is now three and a half and has a younger brother, Benjamin, who was born a few days after his father's death.

The circumstances appear similar to those of the death on Sunday of Miles Frost, 31, the eldest son of the late broadcaster Sir David Frost. Frost, a founding partner of the private equity group Frost-Brooks, collapsed during a run near the family home in Oxfordshire. He was described as a fitness enthusiast who had competed in a half-marathon in a Kenyan game reserve, was careful about his diet and did not drink. The cause of his death is not yet known but it bears many of the hallmarks of sudden arrhythmic death syndrome (SADS), an inherited cardiac condition which is often not revealed until a death occurs in a family.

Dickinson's death was attributed to SADS, which is caused when someone has a ventricular arrhythmia, or a disturbance in the heart's rhythm, that triggers a cardiac arrest even though the stricken person has no heart disease. This cause of death was previously called sudden adult death syndrome but the name was changed because it also affects children.

Every week in the UK at least 12 young people under the age of 35 die suddenly from a previously undiagnosed heart condition. In 80 per cent of those sudden cardiac deaths — 600 a year — there will have been no sign of symptoms.
Nick Rawlinson, a 30-year-old calibration engineer at Jaguar Land Rover, was another extremely fit young man who had no awareness that he had a heart condition. He worked out in the gym most days and had been training for an arduous cycling race in the Italian Dolomites when he failed to turn up for work one day in February 2012. His younger brother, Chris, went to his flat and found him in bed as if he were fast asleep.

Nick Rawlinson had died in his sleep and would not have known anything about it. SADS deaths often occur at night, during sleep, and it is believed that cot death, or sudden infant death syndrome (SIDS) may be partly down to the same causes as SADS.

Post mortem examinations of those under 35 who have died of sudden cardiac arrest divide the fatalities into two groups. Those in the first group are found to have had an underlying structural heart problem that they didn't know about, typically thickening of the heart wall. This is not uncommon: about 1 in 500 of the population may have such a condition.
In the second group — about 5 per cent of those who die suddenly of an unexpected cardiac arrest — no definite cause of death can be found by the cardiac pathologist and sudden arrhythmic death syndrome is then diagnosed.

If toxicology tests reveal that the deceased was clean it is very likely that SADS was the cause. One in three hundred of us harbour the conditions that may open us to the risk of a sudden cardiac arrest, but we may often go through life blissfully unaware of it. "Unfortunately, for some the first symptom is sudden death," says Dr Amanda Varnava, a consultant cardiologist at Imperial College.

Varnava is on the expert panel of cardiologists that advises the Football Association. Conditions that cause SADS seem to be exacerbated by exercise, especially if it is intense. "It is possible to be perfectly fit and healthy and be vulnerable to your heart — out of the blue — suddenly beating very fast," says Varnava. "Athletes are at two to four times greater risk of sudden death because of intense activity."

Fabrice Muamba, the Bolton Wanderers player, collapsed during a 2012 FA Cup match against Tottenham and his heart stopped for 78 minutes. He made a remarkable recovery but his career was over. Muamba may have had Brugada syndrome, one of a group of rare diseases called ion channelopathies that can cause SADS/ arrhythmias. These diseases can be identified by screening (but can only be identified in living people).

In Italy screening is mandatory for all young people playing organised sport and the incidence of sudden cardiac death in the young has been reduced by 90 per cent. Varnava screens many Premiership football players and on four occasions has found players with conditions that left them vulnerable to sudden cardiac arrest. In most cases such conditions end a professional sporting career.

However, non-professional athletes can still play sport, possibly while using beta blockers or after having an implantable cardioverter defibrillator (ICD) fitted, as Muamba had. This is placed under the skin and attached to the heart by electrical leads. It acts like a pacemaker and can detect dangerous arrhythmias and deliver an electric shock to reset the heart. "You can still play sport," says Varnava. "We don't want to convert people who are fit and active to sofa-loving individuals, but for most people the diagnosis rules out competitive sport."

An ECG, or electrocardiogram, will detect most of the conditions, but screening is not infallible. Muamba would have been screened but his condition was not picked up. The NHS has looked at more extensive screening but no testing has yet been devised that is regarded as cost-effective on a large scale. The charity CRY has provided a screening programme for more than 18 years and now tests 15-16,000 young people across the UK every year.

Nick Rawlinson had not had screening for an inherited cardiac condition. After his death his parents and younger brother, Chris, were screened to see if they too had an inherited cardiac condition. Blood relatives are urged to undertake such screening. Chris, 30, took an ECG that prompted his consultant to subject him to a more intrusive electrophysiological study, which unmasked Brugada Syndrome.
He has been fitted with an implantable cardioverter defibrillator, inserted under one of his pectoral muscles. He is able still to go mountain bike riding and snowboarding and fortunately has yet to be delivered of an electric shock by the ICD. The British Heart Foundation warns that without appropriate treatment the outlook for people with Brugada syndrome can be poor.

There are many drugs that should be avoided by those with the syndrome and other inherited cardiac conditions. Nick Rawlinson had epilepsy and it is possible that his medication for that could have played a role in his death.
It is not lost on Chris Rawlinson that without the calamity that befell his brother he would not have been warned of his own condition "unless the same thing had happened to me". Friends and family have set up an annual cycle race in Warwickshire, the Rawlinson Bracket, in memory of Nick and the noisy bottom bracket on his bike. The route traces some of his favourite cycling paths and raises money for SADS research.

Many have called not only for greater screening, but also for better resuscitation facilities so that if a cardiac arrest does occur, individuals are more likely to survive if a bystander performs CPR and uses an external defibrillator. These are increasingly available in public spaces and allow a far greater chance of survival. Members of the public can use this automated equipment to deliver life-saving shock to those in need. Varnava regards it as "disgraceful" that some gyms do not have defibrillators.
Carys Dickinson and her friends have been involved in fundraising for SADS UK, the charity that works to raise awareness of SADS and to fund defibrillators. The charity wants it to be mandatory for all schools to have a defibrillator on the premises.
Andrew Dickinson had not been screened for an inherited cardiac condition.

However, one day, when they are a little older, Henry and Benjamin, the son Andrew never met, will be taken by Carys for tests to establish whether they have inherited the condition that killed their father. Forewarned, and with the right treatment and lifestyle, it need not endanger them.

Sadsuk.org.ukc-r-y.org.uk

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