Heart 2009;95:1646-1647
Editorials
Myocardial fibrosis in thalassaemia: recalling the past or telling the future?
1 CMR Unit, Royal Brompton Hospital, London, UK
2 Imperial College, London, UK
Correspondence to Professor D J Pennell, Royal Brompton Cardiovascular Magnetic Resonance Unit, Sydney Street, London SW3 6NP, UK; d.pennell@imperial.ac.uk
| The first 150 words of the full text of this article appear below. |
"Trust thyself: every heart vibrates to that iron string." Ralph Waldo Emerson
According to WHO data, approximately 56 000 babies are born each year with thalassaemia major, including at least 30 000 who require lifelong transfusions to survive. Of those transfused, under 40% obtain adequate chelation therapy. An estimated 100 000 patients world wide are currently living with regular transfusions, but at least 3000 die each year in their teens or early 20s from uncontrolled iron overload, mainly due to heart failure. With no physiological excretory pathway, iron from transfused blood accumulates in the liver, heart, endocrine and other organs causing tissue damage and impairment in function. Chelating agents can remove the excess iron and prevent complications, the most serious of which is death from heart failure due to myocardial siderosis. Progressive cardiac iron loading eventually leads to left ventricular (LV) dilatation and reduction in LV ejection fraction but this
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Heart 2009 95: 1688-1693.[Abstract] [Full Text] [PDF]
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