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Updated: Jan 23, 2026

Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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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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Discrepancy in patterns of myocardial involvement in beta-thalassaemia vs. sickle cell anaemia

Antoine Fakhry AbdelMassih1, Khaled M Salama2, Carolyne Ghobrial3

  • 1Pediatric Cardiology Unit, Pediatrics' Department, Faculty of Medicine, Cairo University (Kasr Al Ainy), Cairo, Egypt.

Acta Cardiologica
|June 6, 2019
PubMed

Insights

Sickle cell disease (SCD) causes subendocardial dysfunction due to ischemia, while beta thalassaemia major (TM) leads to subepicardial dysfunction from iron overload. These findings highlight differing mechanisms in heart dysfunction for these hemoglobinopathies.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Myocardial dysfunction in sickle cell disease (SCD) and beta thalassaemia major (TM) arises from distinct pathophysiological mechanisms.
  • TM primarily affects the subepicardium via iron overload, whereas SCD impacts the subendocardium through ischemia.

Purpose of the Study:

  • To investigate and compare patterns of left ventricular (LV) dysfunction in patients with SCD and TM.
  • To differentiate between subepicardial and subendocardial dysfunction using advanced echocardiographic techniques.

Main Methods:

  • Conventional echocardiography, 3D echocardiography for ejection fraction, and 2D Speckle tracking myocardial layer strain discriminating echocardiography (MLSD-STE) were employed.
  • Study included 40 TM patients, 40 SCD patients, and 40 age/surface area-matched controls.
  • Biomarkers including hemoglobin, ferritin, and lactate dehydrogenase levels were assessed.

Main Results:

  • TM patients exhibited predominantly subepicardial dysfunction (epicardial GLS: -10.9 ± 2).
  • SCD patients showed prevalent subendocardial dysfunction (endocardial GLS: -10.6 ± 1.6).
  • Statistically significant differences in epicardial and endocardial GLS were observed between TM and SCD groups (p < 0.01).

Conclusions:

  • The study reveals divergent microcirculatory mechanisms underlying myocardial dysfunction in SCD and TM.
  • SCD is associated with subendocardial dysfunction and ischemia, while TM involves iron-induced subepicardial injury.

Keywords:
Subendocardiumhaemoglobinopathiesspeckle tracking echocardiographysubepicardium

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