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Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition

Jane S Hankins1, Mary Beth McCarville, Claudia M Hillenbrand

  • 1Department of Hematology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. jane.hankins@stjude.org

Pediatric Blood & Cancer
|July 27, 2010
PubMed

Insights

Diastolic dysfunction is common in children with sickle cell anemia (SCA) and iron overload. However, this dysfunction is not linked to iron levels in the heart or transfusions, suggesting other disease factors are responsible.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Cardiovascular Imaging

Background:

  • Cardiac failure can result from myocardial iron deposition in patients with transfusion-related iron overload.
  • Iron overload can lead to left ventricular dysfunction in hematologic disorders.
  • Sickle cell anemia (SCA) patients often have diastolic dysfunction, even without transfusions.

Purpose of the Study:

  • To investigate the relationship between transfusional iron burden, myocardial iron deposition, and diastolic ventricular dysfunction in children with SCA.
  • To assess iron overload using T2*-MRI and tissue Doppler echocardiography.

Main Methods:

  • Included children (>=7 years) with SCA and iron overload (serum ferritin >1,000 ng/ml or >=18 lifetime transfusions).
  • Measured serum ferritin, hepatic iron content (HIC), and performed T2*-MRI, echocardiogram, electrocardiogram, and MUGA scan.
  • Compared echocardiographic data with age-matched normative data.

Main Results:

  • 30 children with SCA (median age, 13 years) had elevated HIC and serum ferritin.
  • Mean T2*-MRI was 33 msec.
  • High prevalence of diastolic dysfunction (77% low mitral annular velocity, 45% low tricuspid annular velocity) was observed, but not significantly associated with HIC or T2*-MRI.

Conclusions:

  • Diastolic dysfunction in children with SCA is not associated with transfusional iron burden or myocardial iron deposition.
  • Diastolic dysfunction in SCA is likely due to disease pathophysiology and severity, not iron overload.
Abstract

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