Dr Mary Sheppard: how post mortems benefit the living
The charity Cardiac Risk in the Young (CRY) funds the CRY Centre for Cardiac Pathology (CRY CCP), based at St George’s Hospital in London, which is an international cardiac referral centre for bereaved families who have suffered a sudden cardiac death. It helps families like Samuel's to understand why a loved one has passed away, helping them to avoid unnecessary anxiety and uncertainty.
Cardiac Risk in the Young and the Centre for Cardiac Pathology
CRY CCP’s founding grant in 2008 was thanks to the extraordinary fundraising efforts of a Surrey family who lost both a father and teenage son to sudden cardiac death. Father of three Howard English was 32 when he collapsed and died while playing rugby. Ten years later, his eldest son Sebastian died, also playing rugby, aged just 15. After postmortem investigation, it was found both father and son had suffered from the same genetic condition known as ARVC (arrhythmogenic right ventricular cardiomyopathy).
These cases highlighted the importance of correct pathology after a sudden death, to ensure other family members are appropriately screened and treated by experts. The family and friends of Sebastian and Howard have raised over £700,000 to help offer this service to other families – a service that could have saved Sebastian’s life. The service is led by expert cardiac pathologist, Professor Mary Sheppard, with a team of staff funded by CRY. Young sudden cardiac death is often the first symptom of an undetected cardiac condition, and specialist pathology is required to identify the disease and bring the genetic implications of a potentially inherited disorder to the attention of relatives. When a cause of death is ‘unascertained’ and the deceased is aged 35 or under, the centre will provide a free, fast-track cardiac diagnostic service. CRY aims to remove the unnecessary suffering of the family by reducing the wait to know what has caused the death. The post-mortem examination and report from the centre will usually be completed within two weeks and a significant majority of coroners now routinely refer to this centre when there is a young sudden cardiac (or unascertained) death.
On 21 October 2018, Samuel (Sam) died just before his 28th birthday. He was out running with his wife, Emily, when he collapsed. Paramedics were called and he was ‘shocked’ several times before being taken to St Bartholomew’s Hospital, where doctors were unable to save him. Professor Sheppard was consulted to help understand Sam’s cause of death and, through the postmortem investigations, found that it was due to ARVC. If this diagnosis had not been made by Professor Sheppard and her team, Sam’s family might never have known the cause of death.
Since this is a genetic condition, there is a 50% chance that other blood relatives have the same condition. Sam’s family have now been referred to the CRY Centre for Inherited Cardiovascular Conditions to see if they are at risk too. As well as helping a family understand the cause of death, expert cardiac pathology informs a family whether close blood relatives need to be screened, and also provides vital information for the cardiologist who is screening the family about the type of investigations they will need.
The funding for the centre has allowed more than 2,200 expert cardiac post mortems on young people (aged 35 and under) to have been conducted since the centre was founded. Professor Sheppard and her team have built up the largest database of expert cardiac investigations of young sudden cardiac deaths in the world. This has enabled groundbreaking research that has transformed the understanding of young sudden cardiac death in the UK and internationally.