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Published on: March 14, 2017

Myocardial ischaemia in children with sickle cell disease

M de Montalembert1, C Maunoury, P Acar

  • 1Service de Pédiatrie Générale, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France. mariane.demontal@nck.ap-hop-paris.fr

Archives of Disease in Childhood
|March 23, 2004
PubMed

Insights

Sickle cell disease (SCD) can cause heart problems in children, including reduced blood flow to the heart muscle. Thallium-201 SPECT imaging revealed these issues, which improved with hydroxyurea treatment.

Area of Science:

  • Pediatric Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Sickle cell disease (SCD) can affect the heart through chronic anemia, leading to enlarged left ventricles and potential heart failure.
  • Cardiac involvement in SCD is a significant concern, necessitating investigation into specific complications like myocardial ischemia.

Purpose of the Study:

  • To determine if children with sickle cell disease experience myocardial ischemia.
  • To identify risk factors associated with the development of cardiac ischemia in pediatric SCD patients.

Main Methods:

  • Utilized thallium-201 single-photon emission computed tomography (SPECT) in 22 children with SCD.
  • Patients selected based on chest pain or echocardiographic/ECG findings suggestive of myocardial ischemia.

Main Results:

  • Abnormal myocardial perfusion defects were detected in 14 out of 22 children via SPECT.
  • Perfusion defects were associated with older age and more severe SCD, with some patients experiencing cardiac symptoms.
  • Three patients showed improved myocardial perfusion after six months of hydroxyurea therapy.

Conclusions:

  • Myocardial perfusion defects are a demonstrable complication in children with sickle cell disease.
  • SPECT imaging is effective in detecting these cardiac perfusion abnormalities.
  • Hydroxyurea treatment showed potential for improving myocardial perfusion in pediatric SCD patients.
Abstract

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