Purging iron from the heart.
Methods are now available to measure the magnitude of iron accumulation in the heart. Their validation currently relies on indirect evidence and not on chemical estimation in cardiac biopsies. All patients with symptomatic heart disease appear to have abnormal T2* values, but many patients without symptomatic heart disease also have evidence of increased myocardial iron. Although there is no proof to date that increased myocardial iron, as evidenced by abnormal magnetic resonance imaging, carries an adverse prognosis, it is likely that such new information will affect the chelating programme of patients. In these cases, there are a number of options available: (i) ongoing treatment with either desferrioxamine (DFO) or deferiprone may be intensified; (ii) the patient may be switched to the alternative chelator or (iii) combined chelation with both DFO and deferiprone may be started, which is more effective than using either chelator alone. For patients with symptomatic heart disease, continuous intravenous DFO with, or without deferiprone, remains the currently recommended treatment, in view of its documented ability to salvage these patients.
Hershko
Chaim
C
Hebrew University Hadassah Medical School, Jerusalem and Ben Gurion University Faculty of Medicine, Beer Sheva, Israel. hershko@szmc.org.il
Cappellini
Maria D
MD
Galanello
Renzo
R
Piga
Antonio
A
Tognoni
Gianni
G
Masera
Giuseppe
G
D009202:Cardiomyopathies / Q000175:diagnosis / Q000188:drug therapy*; D002316:Cardiotonic Agents / Q000627:therapeutic use; D000077543:Deferiprone; D003676:Deferoxamine / Q000627:therapeutic use*; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use*; D019190:Iron Overload / Q000175:diagnosis / Q000188:drug therapy*; D009682:Magnetic Resonance Spectroscopy; D011728:Pyridones / Q000627:therapeutic use*
D002316:Cardiotonic Agents; D007502:Iron Chelating Agents; D011728:Pyridones; D000077543:Deferiprone; D003676:Deferoxamine