Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance.
OBJECTIVE
To assess the effects of sickle cell anemia (SCA) on the right ventricle (RV).


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Qureshi
Naveen
N
Department of Pediatrics, David Geffen School of Medicine at UCLA, Torrance and Los Angeles, CA, USA. nqureshi@mail.cho.org
Joyce
James J
JJ
Qi
Ning
N
Chang
Ruey-Kang
RK
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000503:physiopathology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000503:physiopathology*; D017380:Hypertrophy, Right Ventricular / Q000000981:diagnostic imaging / Q000503:physiopathology*; D007223:Infant; D008297:Male; D014655:Vascular Resistance / Q000502:physiology*; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*