Development of thalassaemic iron overload cardiomyopathy despite low liver iron levels and meticulous compliance to desferrioxamine.
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.
Anderson
Lisa J
LJ
Cardiovascular MR Unit, Royal Brompton Hospital, London, UK.
Westwood
Mark A
MA
Prescott
Emma
E
Walker
J Malcolm
JM
Pennell
Dudley J
DJ
Wonke
Beatrix
B
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
D017262:Siderophores; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine