David Frost’s son and hidden heart defects that kill without warning
WHEN sir David Frost’s e l dest s on, Miles, died suddenly while jogging, his family were left with many unanswered questions. The 31-year-old financier collapsed last July near his home in oxfordshire. What made his death so shocking was that he was a keen sportsman and appeared in excellent health and good spirits.
It has now emerged that he suffered from a genetic heart condition, hypertrophic cardiomyopathy (HCM).
The condition, which causes an abnormal thickening of the walls of the heart, can lead to sudden death at any age — though proper treatment can control symptoms and prevent complications. It is passed on through families and — as the Frost family revealed this weekend — sir David, who died of a heart attack in 2013, had HCM. This isn’t what caused the broadcaster’s death, but emerged during his post-mortem examination.
If a parent has the condition, there is a 50 per cent chance their child will inherit it, and it is a red-flag warning sign. But Miles’s heart condition was not identified until after his death.
sir David’s surviving sons, George and Wilfred, have said neither they nor their late brother were told of the risk that they could have inherited the condition.
‘sadly, this was not flagged up to us; thus Miles, George and I were not tested two years ago, as we should have been,’ Wilfred said. ‘Clearly, this adds to the agony relating to Miles’s death.’
If someone is diagnosed with the condition or dies from it, immediate family members should be referred for clinical and genetic testing (known as cascade testing). But currently there is no obligation for coroners to refer relatives for testing when HCM is spotted in postmortem examinations, and no coordination of services nationally.
The Frost brothers say the condition ‘ can remain unknown, then strike suddenly. This is often, though not always, i n young people who push their bodies through sport and exercise, which is what happened to Miles.’
At least 12 otherwise healthy young people, aged between 14 and 35, die suddenly every week in the UK. In most cases, their deaths are caused by an undiagnosed heart condition, which triggers a fatal cardiac arrest — where the heart stops beating because of an THIs electrical problem.
usually occurs after puberty — when the heart has fully developed — and under t he strain of strenuous exercise.
Alarmingly, one in 300 people in this age group has an underlying heart abnormality that puts them at increased risk of cardiac arrest, according to charity Cardiac Risk in the Young (CRY). Yet few are diagnosed because in 80 per cent of cases there are no symptoms.
Fiona Barnes’s world was torn apart when her boyfriend, James Phillips, died while taking part in the 2014 Reigate half-marathon.
James, a 27-year- old architect from Denham, Buckinghamshire, collapsed 200 metres from the finish line. Tests after his death revealed there was nothing wrong — it was recorded as sudden Arrhythmic Death syndrome, the generic term for a death caused by a heart problem without a definite cause (an electrical fault in the heart is often the cause, but such faults can only be picked up when the heart is beating).
‘James was fit and active with no signs of health problems,’ says 28-year- old Fiona, who is from Amersham, Buckinghamshire.
‘I had just finished running the half-marathon when I got a call to say he had collapsed.
‘ He j ust f ell to the ground suddenly. Friends who are nurses were giving him CPR within seconds and paramedics took over when they arrived.’
seventeen months since James’s death, Fiona, who works in human resources at the BBC, is still in shock. ‘It has obliterated my life,’ she says.
Inherited heart conditions are the most common cause of sudden death in young people, says Dr Aneil Malhotra, a cardiologist at st George’s Hospital, London.
They f al l i nto t wo broad categories: cardiomyopathies — diseases that affect the heart muscle, the most common being hypertrophic cardiomyopathy — and electrical disturbances such as Brugada or long QT syndromes, which can make the heart’s rhythm so erratic it goes into a cardiac arrest.
‘In people with HCM, the muscle fibres of the heart are tangled when you look at them under the microscope,’ says Dr Malhotra. ‘This makes the walls of the heart thicker, eventually making it harder for it to pump blood.
‘While this may not have much of a detrimental effect while resting, when the heart is working harder during exercise and the electrical pathways that run between the muscles in the heart are put under strain, it increases the risk of cardiac arrest.
‘If you have this condition and are usually fit and active, you are three times more likely to have a cardiac arrest while exercising than other people.’
With Brugada and long QT, the heart l ooks normal but its electrical pathways — which regulate beating — are abnormal, again increasing the risk of cardiac arrest, often during exercise.
‘Inherited heart conditions are much more l i kely to cause a problem in young people because they are more active, and exercise can place extra strain on their hearts,’ adds Dr Malhotra, who is also a spokesperson for CRY.
Warning symptoms that should be checked out with a doctor i nclude: chest pain, unusual s hortness of breath, heart palpitations, dizziness and loss of consciousness or fainting when exercising, and a family history of heart conditions (including the death of a relative under the age of 50 of a heart condition).
so can these deaths be prevented i f most people with t hese conditions have no symptoms?
In many cases, screening picks up such heart conditions, and there are now specialist clinics for inherited heart problems in most major cities, which can carry out tests including electrocardiograms ( ECG) to l ook at the electrical activity of the heart.
The ECG is 90 per cent accurate at identifying hypertrophic cardiomyopathy, says Dr Malhotra.
Top athletes and family members of those who have died suddenly should be offered these tests, as they may be at higher risk.
Those who don’t have symptoms or a family link can still be screened — CRY recommends this to those aged between 14 and 35, and it runs free mobile screening clinics.
Alternatively, private screening can be arranged through CRY for HOWEVER, a sports club, for example.
underlying heart conditions don’t al ways s how up in screening tests. There is no cure for them — Dr Malhotra explains that treatment is about mitigating the risks. Patients with HCM are advised to do only light to moderate exercise and to avoid sports such as football and basketball, where there are lots of starts and stops, which cause adrenaline rushes.
Medication such as betablockers, which slow down the heart, can also help, and some patients have a defibrillator implanted in the chest to kickstart the heart if it stops beating.
After James’s death, Fiona Barnes had her heart checked and she now encourages others to do the same. ‘ Everyone thinks it won’t happen to them or their friends or children, but it does,’ she says.
Meanwhile, George and Wilfred Frost have launched the Miles Frost Fund in partnership with the British Heart Foundation.
It will fund specialised genetic nurses and counsellors to ensure that GPs, cardiologists and coroners are all referring at-risk patients for cascade testing. It also aims to build programmes of cascade testing across the UK.
FOR more, see bhf.org.uk/miles-frost-fund and testmyheart.org