title: Myocardial iron loading in patients with thalassemia major on deferoxamine chelation.
abstract: BACKGROUND
  Heart failure secondary to myocardial iron loading remains the leading cause of death in thalassemia major (TM). We used cardiovascular magnetic resonance (CMR) to assess the prevalence of myocardial iron overload and ventricular dysfunction in a large cohort of TM patients maintained on conventional chelation treatment with deferoxamine.
  
  
  METHODS
  A mobile CMR scanner was transported from London, UK, to Sardinia, Italy where 167 TM patients were assessed for myocardial iron loading, B-natriuretic peptide (BNP), and ferritin. In patients with myocardial iron loading CMR assessments of ventricular function were also made.
  
  
  RESULTS
  Myocardial iron loading (T2* < 20 ms) was present in 108 (65%) patients, which was severe (T2* < 8 ms) in 22 (13%). Impaired (< 56%) left ventricular (LV) ejection fraction (EF) was present in 5%, 20% and 62% of patients with mild, moderate or severe iron loading. Increasing myocardial iron was related to impaired LVEF (Rs = 0.57, p < 0.001), weakly related to serum ferritin (Rs = -0.34, p < 0.001), and not related to liver iron (Rs = 0.11, p = 0.26). BNP was weakly related to myocardial iron (Rs = -0.35, p < 0.001) and was abnormal in only 5 patients.
  
  
  CONCLUSIONS
  Myocardial siderosis was found in two-thirds of thalassemia major patients on maintenance deferoxamine treatment. This was combined with a high prevalence of impaired LV function, the severity of which tracked the severity of iron deposition. BNP was not useful to assess myocardial siderosis.
authors.affiliation: Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom.
authors.forename: M A
authors.initials: MA
authors.lastname: Tanner
authors.forename: R
authors.initials: R
authors.lastname: Galanello
authors.forename: C
authors.initials: C
authors.lastname: Dessi
authors.forename: M A
authors.initials: MA
authors.lastname: Westwood
authors.forename: G C
authors.initials: GC
authors.lastname: Smith
authors.forename: S V
authors.initials: SV
authors.lastname: Nair
authors.forename: L J
authors.initials: LJ
authors.lastname: Anderson
authors.forename: J M
authors.initials: JM
authors.lastname: Walker
authors.forename: D J
authors.initials: DJ
authors.lastname: Pennell
mesh_terms: D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology; D016009:Chi-Square Distribution; D003676:Deferoxamine / Q000627:therapeutic use*; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D019190:Iron Overload / Q000175:diagnosis* / Q000453:epidemiology; D007558:Italy / Q000453:epidemiology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D009206:Myocardium / Q000737:chemistry* / Q000378:metabolism; D020097:Natriuretic Peptide, Brain / Q000097:blood; D015995:Prevalence; D017262:Siderophores / Q000627:therapeutic use*; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis / Q000453:epidemiology; D017086:beta-Thalassemia / Q000188:drug therapy*
chemical_list: D017262:Siderophores; D020097:Natriuretic Peptide, Brain; D005293:Ferritins; D003676:Deferoxamine