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Arch Dis Child 2004;89:359-362 doi:10.1136/adc.2003.027326
  • Acute paediatrics

Myocardial ischaemia in children with sickle cell disease

  1. M de Montalembert1,
  2. C Maunoury2,
  3. P Acar3,
  4. V Brousse1,
  5. D Sidi3,
  6. G Lenoir1
  1. 1Service de Pédiatrie Générale, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France
  2. 2Service de Médecine Nucléaire, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France
  3. 3Service de Cardiologie Pédiatrique, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France
  1. Correspondence to:
    Dr M de Montalembert
    Service de Pédiatrie Générale, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France; mariane.demontalnck.ap-hop-paris.fr
  • Accepted 27 July 2003

Abstract

Background: The heart may be involved in children affected with sickle cell disease (SCD) via several mechanisms. Principally, chronic anaemia increases cardiac output and may cause left ventricular enlargement and cardiac insufficiency.

Aims: To investigate whether the heart also suffers from ischaemia in SCD, as has already been shown for other organs (bone, brain, etc), and to look for risk factors predisposing to this complication.

Methods: Twenty two children with SCD, and chest pain or ECG or echocardiographic signs (left ventricle dilation or hypokinesis) suggesting myocardial ischaemia were subjected to thallium-201 (201Tl) single photon emission computed tomography (SPECT).

Results: Eight children had a normal SPECT, 14 an abnormal one. Myocardial perfusion defects were reversible in nine, fixed in five. Patients with perfusion defects tended to be older and have more severe disease. Five had had cardiac symptoms (episodes of cardiac failure in three, ventricular fibrillation in one, angina in one). Myocardial perfusion was reassessed after six months of hydroxyurea treatment in three patients, and was found to be improved.

Conclusions: Myocardial perfusion defects are present in children with SCD and may be demonstrated using SPECT. Hydroxyurea improved perfusion in three patients.

Footnotes

    Responses to this article

    • Myocardial ischaemia in Sickle Cell Disease
      • Athanassios Aessopos,
      • Maria Tsironi
      ADC published online May 21, 2004

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