title: Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy.
abstract: OBJECTIVE
  To use cardiovascular magnetic resonance (CMR) techniques to examine possible causes for the left ventricular (LV) dilatation that occurs in sickle cell disease (SCD), including the effects of chronic anemia, iron-induced cardiomyopathy, and regional fibrosis due to sludge infarcts that occur during sickle crises.
  
  
  METHODS
  A total of 47 patients with sickle cell anemia were assessed for LV function and myocardial iron levels using CMR measurements; 30 of these were also assessed for regional fibrosis using late gadolinium-enhancement CMR. The LV function was compared to both normal controls and transfusion dependent non-iron-loaded (NIL) thalassemia major (TM) patients.
  
  
  RESULTS
  Only one SCD patient had significant myocardial iron loading, and only two patients had regional fibrosis. There were significant differences in ventricular volumes of the sickle patients compared with both the normal controls and the NIL-TM population (P < 0.01).
  
  
  CONCLUSIONS
  The LV changes seen in SCD are partly the result of a chronic anemia but there appears to be another contributory factor. This extra factor is not myocardial iron loading or regional fibrosis, although a homogenous fibrotic disorder affecting the left ventricle cannot be excluded.
authors.affiliation: Cardiovascular MR Unit, Royal Brompton Hospital and Imperial College, London, UK.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Westwood
authors.forename: Farrukh
authors.initials: F
authors.lastname: Shah
authors.forename: Lisa J
authors.initials: LJ
authors.lastname: Anderson
authors.forename: Julian W
authors.initials: JW
authors.lastname: Strange
authors.forename: Mark A
authors.initials: MA
authors.lastname: Tanner
authors.forename: Alicia M
authors.initials: AM
authors.lastname: Maceira
authors.forename: Jo
authors.initials: J
authors.lastname: Howard
authors.forename: John B
authors.initials: JB
authors.lastname: Porter
authors.forename: J Malcolm
authors.initials: JM
authors.lastname: Walker
authors.forename: Beatrix
authors.initials: B
authors.lastname: Wonke
authors.forename: Dudley J
authors.initials: DJ
authors.lastname: Pennell
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000473:pathology*; D001803:Blood Transfusion; D009202:Cardiomyopathies / Q000175:diagnosis / Q000473:pathology*; D005260:Female; D005682:Gadolinium / Q000494:pharmacology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D017086:beta-Thalassemia / Q000473:pathology
chemical_list: D005682:Gadolinium