Myocardial ischaemia in children with sickle cell disease

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 (Tl-201) 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.