Right ventricular abnormalities in sickle cell anemia: Evidence of a progressive increase in pulmonary vascular resistance

To assess the effects of sickle cell anemia (SCA) on the right ventricle (RV). Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index, fractional area change, free-wall mass index, ejection time corrected for heart rate (ET c), and tricuspid regurgitation (TR) gradient. SCA subjects had elevated RV ETc (mean ± standard deviation, 0.369 ± 0.030 sec vs 0.351 ± 0.022 sec; P < .01), diastolic area index (19.9 ± 2.4 cm 2/m 2 vs 13.2 ± 2.1 cm 2/m 2; P < .01) and free-wall mass index (33.2 ± 4.4 g/m 2 vs 23.9 ± 4.3 g/m 2; P < .01), whereas RV fractional area change (37 ± 8% vs 36 ± 4%) was not different from controls. Although RV diastolic area index in SCA paralleled the normal range over time, RV free-wall mass index continued to gradually rise throughout childhood ( r = .42; P < .05). TR gradients > 2.5 m/sec, consistent with pulmonary hypertension, were found in 5 (16%) of SCA subjects, all older than 9 years. RV preload and systolic function do not worsen during childhood in SCA; however, RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.