title: Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition.
abstract: BACKGROUND
  Cardiac failure from myocardial iron deposition is a severe complication in patients with transfusion-related iron overload. Progressive heart damage from iron overload can cause left ventricular systolic and diastolic dysfunction in patients with hematologic disorders. Since nontransfused patients with sickle cell anemia (SCA) have a high incidence of diastolic dysfunction, we investigated the relationships among transfusional iron burden, myocardial iron deposition, and diastolic ventricular dysfunction by T2*-MRI and tissue Doppler echocardiography in iron-overloaded children with SCA.
  
  
  METHODS
  Children (> or =7 years) with SCA and iron overload (serum ferritin >1,000 ng/ml or > or =18 lifetime transfusions) were eligible. Serum ferritin and hepatic iron content (HIC) were measured and participants underwent nonsedated T2*-MRI of the heart, echocardiogram, electrocardiogram, and multi-uptake gated acquisition (MUGA) scan. Age-matched normative echocardiographic data were used for comparison.
  
  
  RESULTS
  Among 30 children with SCA (median age, 13 years) and iron overload, mean (+/-SD) HIC and serum ferritin were 10.8 mg Fe/g (+/-5.9 mg Fe/g) and 3,089 ng/ml (+/-2,167 ng/ml), respectively. Mean T2*-MRI was 33 msec (+/-7 msec, range, 22-49). Echocardiography showed a high prevalence of diastolic dysfunction (77% and 45% abnormally low mean mitral annular velocity and mean tricuspid annular velocity, respectively); however, echocardiogram and MUGA scan findings were not significantly associated with HIC or T2*-MRI.
  
  
  CONCLUSIONS
  Diastolic dysfunction is not associated with transfusional iron burden or myocardial iron deposition among children with SCA. Diastolic dysfunction likely results from disease pathophysiology and severity rather than iron overload.
authors.affiliation: Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. jane.hankins@stjude.org
authors.forename: Jane S
authors.initials: JS
authors.lastname: Hankins
authors.forename: Mary Beth
authors.initials: MB
authors.lastname: McCarville
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Hillenbrand
authors.forename: Ralf B
authors.initials: RB
authors.lastname: Loeffler
authors.forename: Russell E
authors.initials: RE
authors.lastname: Ware
authors.forename: Ruitian
authors.initials: R
authors.lastname: Song
authors.forename: Matthew P
authors.initials: MP
authors.lastname: Smeltzer
authors.forename: Vijaya
authors.initials: V
authors.lastname: Joshi
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D002648:Child; D003971:Diastole; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D005293:Ferritins / Q000097:blood; D015637:Gated Blood-Pool Imaging; D006801:Humans; D019190:Iron Overload / Q000150:complications*; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000378:metabolism*; D013318:Stroke Volume; D065227:Transfusion Reaction; D018487:Ventricular Dysfunction, Left / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism
chemical_list: D005293:Ferritins