12 April 2011
Researchers from the National Institute for Health Research (NIHR) Cardiovascular Biomedical Research Unit (BRU) at Royal Brompton Hospital have pinpointed the trigger for fatal heart failure in patients living with one of the world’s most common genetic diseases.
The 10-year study of hearts from all over the world has identified high iron levels, resulting from frequent blood transfusions, as the trigger for fatal heart failure in patients with thalassaemia major.
Thalassaemia, which has numerous forms, is an inherited blood disorder affecting around three per cent of the world’s population, mostly of Mediterranean, Middle Eastern, South Asian and African descent. The condition causes defects in haemoglobin production, resulting in anaemia and, for those patients with thalassaemia major, requiring frequent and regular blood transfusions. These transfusions can lead to iron collecting in the heart and triggering heart failure. Past figures showed that around 50 per cent of thalassaemia major patients in the UK died by the age of 35.
The expert team at the BRU, a collaboration with Imperial College London, has used cardiovascular magnetic resonance (CMR) scanning to measure the build-up of iron in the heart and reveal the levels of iron causing heart failure. The study’s results will provide a vital tool for developing new drug treatments.
Leading the study, Professor Dudley Pennell, director of the NIHR Cardiovascular Biomedical Research Unit and the CMR unit at Royal Brompton Hospital, 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 to heart failure and develop drugs that 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 BRU. Applying this research could eradicate heart failure caused by this genetic disease.”
The results of the study from the team at Royal Brompton and Imperial College London, are published today (12 April 2011) in the international journal Circulation[1].
Read our press release about this thalassaemia research.
[1] Circulation – Journal of the American Heart Association.
