Myocardial performance in hydroxyurea versus transfusion treated sickle cell disease pediatric cases

ABSTRACT

Sickle cell disease [SCD] is associated with many cardiac abnormalities including increase cardiac output, pulmonary hypertension [PH] and myocardial perfusion abnormalities. Hydroxyurea [HU] is considered to be the most successful drug therapy for severe sickle cell disease. The objective is to study the echocardiographic cardiac abnormalities in patients with SCD on HU therapy comparing them with those on isolated blood transfusion regimen. Descriptive cross sectional case study was conducted on 29 cases of SCD on transfusion regimen [16 cases] and on HU therapy [13 cases]. Doppler echocardiographic assessment of left ventricular systolic and diastolic function, calculation of myocardial performance index [MPI] and left ventricular mass were done to all patients. Right ventricular MPI was higher in HU treated group with difference approaching significance [p = 0.06]. No significant correlation between HU dose and duration and left ventricular systolic or diastolic function, right ventricular diastolic function, or pulmonary artery pressure [PAP]. PH was present in 55% of SCD. No significant difference in PAP between HU and transfusion treated groups. Negative significant correlation between left ventricular deceleration time and estimated systolic pulmonary artery pressure [r=-0.37 p =0.04]. No deleterious echocardiographically detected cardiac effects were observed in HU treated SCD patients. HU had no effect on PH