29 March 2018
Experts at Royal Brompton & Harefield NHS Foundation Trust are to be at the forefront of a £2 million study into the potentially deadly heart condition dilated cardiomyopathy (DCM).
After coronary heart disease, DCM is the most common cause of heart failure and carries a risk of sudden death. It is estimated that it affects up to a quarter of a million people in UK and is the most common reason patients require heart transplants.
The causes for most cases of DCM are unknown and, at the moment, there are poor outcomes for patients. Research suggests that up to half of deaths occur within the first two years of diagnosis and 15 per cent of patients do not survive beyond five years after diagnosis.
The condition stretches and thins the heart muscle so it becomes ‘baggy’ and is unable to pump blood around the body efficiently.
For some people with DCM, there is a known genetic cause, but researchers want to find out more about the genes involved, as well as discovering how lifestyle and environmental factors impact the condition.
The study is being funded by the British Heart Foundation and led by Professor Stuart Cook, professor of clinical and molecular cardiology at Imperial College London, along with primary investigators Dr James Ware, honorary consultant cardiologist, and Dr Sanjay Prasad, consultant in cardiology and cardiovascular magnetic resonance, at Royal Brompton Hospital.
Royal Brompton and Harefield hospitals care for a large number of patients with cardiomyopathies and a joint research venture with Imperial College London in 2015 was responsible for identifying a mutation in the Titin gene as the most common genetic cause of DCM.
Professor Cook said: “We urgently need to find out more about what causes DCM, and identify who is at risk of sudden death or developing heart failure.
“For about one in four patients with DCM we can find a genetic cause, but that leaves us with hundreds of thousands of people with DCM that we cannot explain, which hinders our ability to diagnose and treat them or help their families.
“There are currently no targeted treatments that are specific for DCM, but as we get a better understanding of the genes which cause the condition, we can hope to develop new treatments which target these genes and pathways.”
Dr James Ware added:
“This BHF-funded study is bringing together clinical experience and expertise from around the country, including cardiomyopathy teams looking after patients with DCM in the clinic, experts in cardiac magnetic resonance (CMR) imaging, and experts in genetics and genomics.
“We care for a large population with cardiomyopathies at Royal Brompton and Harefield hospitals: an area of great clinical need and a research priority for us. About half of the patients involved in the study have already been recruited, and half will be collected across six other centres from around the country who have come together for this study.”
It is believed that as many as 650,000 (1 in 100) people in the UK may be at risk of DCM due to the common mutation in the titin protein. This mutation means the heart is more likely to develop the condition when it is placed under stress such as pregnancy, some cancer treatments or drug and alcohol use.
The Trust provides a comprehensive inherited cardiac conditions (ICC) service covering adults, children and fetal life. The services include: cardiomyopathy; channelopathy; genetics; aortopathy; familial hypercholesterolaemia and pulmonary hypertension.