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.