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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Myocardial iron loading in patients with thalassemia major on deferoxamine chelation

M A Tanner1, R Galanello, C Dessi

  • 1Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom.

Journal of Cardiovascular Magnetic Resonance : Official Journal of the Society for Cardiovascular Magnetic Resonance
|June 8, 2006
PubMed

Insights

Myocardial iron overload is common in thalassemia major patients on deferoxamine, leading to impaired heart function. B-natriuretic peptide is not a reliable indicator of this condition.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Thalassemia major (TM) patients face high mortality from heart failure due to iron overload.
  • Conventional chelation therapy with deferoxamine is standard care for TM.
  • Cardiovascular magnetic resonance (CMR) is crucial for assessing cardiac complications in TM.

Purpose of the Study:

  • To determine the prevalence of myocardial iron overload in TM patients on deferoxamine.
  • To evaluate ventricular dysfunction in relation to iron loading.
  • To assess the utility of B-natriuretic peptide (BNP) in this cohort.

Main Methods:

  • A cohort of 167 TM patients was assessed using a mobile CMR scanner.
  • Myocardial iron loading was quantified using T2* sequences.
  • Ventricular function (left ventricular ejection fraction) and serum markers (ferritin, BNP) were measured.

Main Results:

  • 65% of patients had myocardial iron loading, with 13% having severe iron.
  • Impaired left ventricular ejection fraction (LVEF) was observed in 5-62% of patients, correlating with iron severity.
  • Myocardial iron correlated with LVEF and serum ferritin but not liver iron; BNP showed weak correlation and was abnormal in few patients.

Conclusions:

  • Two-thirds of TM patients on deferoxamine exhibit myocardial siderosis.
  • Impaired LV function is prevalent and directly related to the degree of myocardial iron deposition.
  • BNP is not a useful biomarker for assessing myocardial siderosis in TM.
Abstract

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