title: Development of thalassaemic iron overload cardiomyopathy despite low liver iron levels and meticulous compliance to desferrioxamine.
abstract: It is believed that myocardial iron deposition and the resultant cardiomyopathy only occur in the presence of severe liver iron overload. Using cardiovascular magnetic resonance, it is now possible to assess myocardial and liver iron levels as well as cardiac function in the same scan, allowing this supposition to be examined. We describe a patient with progressive myocardial iron deposition and the development of early iron overload cardiomyopathy despite excellent compliance to standard subcutaneous desferrioxamine, minimal liver iron and well-controlled serum ferritin levels. These indirect markers remained far below the thresholds conventionally believed to be associated with increased cardiac risk.
authors.affiliation: Cardiovascular MR Unit, Royal Brompton Hospital, London, UK.
authors.forename: Lisa J
authors.initials: LJ
authors.lastname: Anderson
authors.forename: Mark A
authors.initials: MA
authors.lastname: Westwood
authors.forename: Emma
authors.initials: E
authors.lastname: Prescott
authors.forename: J Malcolm
authors.initials: JM
authors.lastname: Walker
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
authors.forename: Beatrix
authors.initials: B
authors.lastname: Wonke
mesh_terms: D009202:Cardiomyopathies / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D002648:Child; D003676:Deferoxamine / Q000008:administration & dosage*; D005293:Ferritins / Q000097:blood; D006321:Heart / Q000000981:diagnostic imaging; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D008099:Liver / Q000000981:diagnostic imaging / Q000378:metabolism*; D008297:Male; D009206:Myocardium / Q000378:metabolism; D011859:Radiography; D017262:Siderophores / Q000008:administration & dosage*; D016276:Ventricular Function / Q000187:drug effects; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging
chemical_list: D017262:Siderophores; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine