Evaluation of left ventricular diastolic function in pediatric sickle cell disease patients

Although cardiac involvement is common in adult sickle cell disease (SCD) patients, it is unknown when cardiac dysfunction appears in the course of SCD. Diastolic function has not been comprehensively analyzed in children with SCD. Thus, our aim was to evaluate diastolic function in pediatric SCD patients. Echocardiograms were performed in 156 (73 SC and 81 control) patients. Left ventricular and left atrial volumes, left ventricular mass, mitral inflow, and tissue Doppler indices (TDI) were obtained. The right ventricular pressures (RVP) were calculated. For each SCD patient, mean hemoglobin (Hb), fetal hemoglobin (HbF) levels, and blood transfusion encounters were recorded. Data were analyzed using t-test, univariate and multivariate regression, and Spearman correlation analyses. SCD patients had significantly larger left ventricular and atrial volumes than controls. Mitral inflow velocities were significantly higher in the SCD group. Of 73 SCD subjects, 32 had left ventricular dilatation, 11 had hypertrophy, and 25 had elevated RVP. Left ventricular size was inversely related to Hb, but unrelated to HbF or age. The early mitral inflow velocity was correlated negatively with Hb and positively with left ventricular sizes. TDI indices did not differ between the SCD and control groups. They were unrelated to Hg, HgF, transfusion history, or ventricular size; however, Doppler indices of left ventricular stiffness were increased in SCD patients. Pediatric SCD patients demonstrate increased left ventricular stiffness, and a significant percentage of them have left ventricular hypertrophy. There are strong positive relations between left ventricular hypertrophy and right ventricular pressure in these patients. TDI should be employed in the evaluation of SCD patients.