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Trinity is helping to promote new research funded by the charity Cardiac Risk in the Young (CRY) which is set to transform the way experts understand conditions that can cause young sudden cardiac death.
CRY believes – as it marks 10 years since the launch of its pioneering Centre for Cardiac Pathology (CCP) – that a continued improvement in the understanding of the conditions that can cause a sudden death (and, if necessary, a re-naming or re-classification of the syndrome) is vital in its quest to prevent these tragedies from occurring.
It also hoped that such breakthroughs in research will help to assure bereaved families that all steps are being taken to identify (and treat) similar risk in surviving siblings, parents and other close relatives.
The new paper, which studied 5,205 sudden cardiac deaths evaluated by Professor Mary Sheppard over a 24-year period, was published last week ahead-of-print in the prestigious international journal Circulation.
In 2008, Prof Sheppard was named Director of CRY’s Cardiac Pathology Centre – originally based at The Royal Brompton, and then relocating 4 years later to its current home at St George’s, University of London
Led by Dr Chris Miles, the new paper reported on 202 post-mortem cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosed at CRY’s CCP. In 87% of cases, there was also involvement of the left ventricle, supporting claims that ARVC is mostly a biventricular disease. The paper also proposed criteria for the pathological diagnosis of arrhythmogenic cardiomyopathy and identified disease-causing genetic mutations in 25% of sudden death cases that underwent testing.
ARVC is a rare inherited heart condition that has long been recognised as a significant (but hidden) cause of sudden cardiac death, particularly among young athletes.
The study also showed that 20% of ARVC sudden death victims were engaged in competitive sport, principally high intensity sports such as football. They were also far more likely to have died during physical exertion.
The CRY CCP was founded thanks to a significant grant from the family of father and son, Howard and Sebastian English. Both died from ARVC, both were playing rugby – yet their deaths occurred 11 years apart.
The link had never been made until Sebastian’s tragic death in 2004, aged just 15. Had Howard’s death been correctly identified, there is every chance that the death of his eldest son could have been prevented.
Sebastian’s mum, Stephanie Hunter, commented; “In 2004, CRY listened and reacted to our family’s plea that multiple deaths in families with an inherited heart condition could not be allowed to continue. The CRY Pathology Centre was set up in Howard and Sebastian’s name, funded by the generosity of our incredible friends and the continued support of CRY patrons, especially Simon Halliday and John Inverdale.
“Howard played for Esher RFC and Sebastian, Haslemere RFC and both clubs have also been so supportive, helping us significantly raise awareness of CRY screening and in our fundraising efforts”.
“This is now the leading cardiac pathology unit in the country and its existence is saving, and has saved, many lives in those families who have suffered the loss of a family member through sudden death with a previously undiagnosed cardiac condition.
“Our family, like many, are so grateful to CRY for existing. We would have been alone and lost without them.”
The “Howard and Sebastian English Memorial Fund” has since raised well over £650,000 to continue supporting the CRY Centre for Cardiac Pathology and to improve understanding of ARVC and how deaths can be avoided. In 1995, it was believed that ARVC affected 1 in 5-7 million of the population – the figure is now known to be closer to 1 in 2-3000.
Dr Steven Cox, Chief Executive of CRY, adds; “Not only is Chris’ research highlighting a greater understanding of this devastating condition that can suddenly strike so many talented athletes at the prime of their lives, but pre-participation screening for conditions such as ARVC is being increasingly recommended to help detect the condition in asymptomatic patients.
“Greater recognition and understanding of these conditions which can have such a horrendous impact if left undiagnosed is something that families connected to CRY are continually fighting for.”