Tuesday 12 April, 2011
Experts from the National Institute for Health Research (NIHR) cardiovascular Biomedical Research Unit at Royal Brompton Hospital and Imperial College London have pinpointed the trigger for fatal heart failure in patients living with one of the most common genetic diseases worldwide. Around 50 per cent of thalassaemia (major) patients in the UK died by the age of 35 in the year 2000. Death at a young age is related to iron collecting in the heart from blood transfusions. The study reveals the amount of iron, which leads to fatal heart failure, providing a vital tool for developing new drug treatments.
Around 60,000 children are born with thalassaemia major every year, which causes defects in haemoglobin production, resulting in anaemia. In its most serious form it can cause organ damage, restricted growth, liver disease, heart failure and ultimately death. Those with the condition need extensive medical care which includes frequent blood transfusions. A major side effect of these transfusions is a build-up of potentially life-threatening iron levels in the heart. This can lead to serious, and in many patients, fatal, heart failure.
Results published in the international journal Circulation on Tuesday (April 12), from the team at Royal Brompton and Imperial College London, show how by using cardiovascular magnetic resonance (CMR) scanning, they have identified the levels of iron which trigger fatal heart failure.
Leading the study, Professor Dudley Pennell, director of the NIHR cardiovascular Biomedical Research Unit and the CMR unit at Royal Brompton Hospital and from the National Heart and Lung Institute at Imperial College London, said: “This is a major breakthrough for thalassaemia patients, as it validates the essential building block for developing new drugs. We can now pinpoint exact levels of iron in the heart, relate this toheart failure and develop drugs which will eradicate this dangerous side-effect of frequent blood transfusions.
“Translating research into better care and treatment, so patients can enjoy the best possible quality of life is the essence of what we do here through the biomedical research unit. Applying this research could eradicate heart failure caused by this genetic disease.”
The team adopted the technique pioneered at Royal Brompton, using cardiovascular magnetic resonance (CMR) to measure the build up of iron in the heart. Identifying the amount of iron which triggers heart failure will help in clinical trials for drugs to treat thalassaemia. In a previous study, experts showed how using cardiovascular magnetic resonance (CMR) to predict future onset of heart failure is more accurate than other methods, such as biopsies or blood tests.
Professor Dame Sally Davies, Chief Medical Officer and Chief Scientific Adviser at the Department of Health said: “This is precisely the sort of practical research with direct patient benefit that the National Institute for Health Research is keen to fund. It is an impressive example of finding new ways to treat some of the most common diseases, for the benefit of patients not just in England but around the world.”
The study looked at hearts donated by patients from around the world over the past decade.
 Circulation – Journal of the American Heart Association.
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Royal Brompton & Harefield NHS Foundation Trust
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Notes to editors:
Thalassaemia is most common in people of Mediterranean, Middle Eastern, Southern Asian and African descent. There are approximately 800 sufferers in the UK.
The research paper On T2* Magnetic Resonance and Cardiac Iron will be published in Circulation on April 12, 2011, 00.01.
Royal Brompton & Harefield NHS Foundation Trust is a national and international specialist heart and lung centre based in Chelsea, London and Harefield, Middlesex. The Trust helps patients from all age groups who have heart and lung problems and is the country's largest centre for the treatment of adult congenital heart disease.
The National Institute for Health Research provides the framework through which the research staff and research infrastructure of the NHS in England is positioned, maintained and managed as a national research facility. The NIHR provides the NHS with the support and infrastructure it needs to conduct first-class research funded by the Government and its partners alongside high-quality patient care, education and training. Its aim is to support outstanding individuals (both leaders and collaborators), working in world class facilities (both NHS and university) and conducting leading edge research focused on the needs of patients. http://www.nihr.ac.uk/
The NIHR Cardiovascular Biomedical Research Unit at Royal Brompton Hospital and Imperial College London is one of 16 Biomedical Research Units (BRUs) established within leading NHS/University partnerships to drive innovation and translational research in biomedicine into NHS practice. Funding was awarded to these BRUs following an NIHR call in 2007.
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