Myocardial perfusion in children with sickle cell anaemia

Background. Myocardial ischaemia is an unexpected complication with potentially serious clinical damages in patients with sickle cell anaemia (SCA). Conventional techniques, such as exercise testing and echocardiography, have low sensitivity and specificity for the detection of myocardial ischaemia in patients with SCA. Objective. To assess myocardial perfusion using thallium-201 (201Tl) single-photon emission computed tomography (SPECT) in children with SCA. Materials and methods. Eight patients (11.5 ± 5.0 years, mean ± SD) who were free of cardiac symptoms were studied. Myocardial perfusion was assessed by 201Tl-SPECT after stress and 3 h later after a further injection. Left ventricular ejection fraction (LVEF) was assessed by equilibrium radionuclide angiography. Results. Myocardial perfusion was abnormal in three of eight patients: two had reversible defects and one had a fixed defect. The mean LVEF was 53 ± 8 %. There was no relationship between myocardial perfusion and LVEF. Conclusions. Treatment of asymptomatic myocardial ischaemia remains unclear, and more aggressive therapy of the haematological disease should be considered.