title: Right ventricular abnormalities in sickle cell anemia: evidence of a progressive increase in pulmonary vascular resistance.
abstract: 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.
authors.affiliation: Department of Pediatrics, David Geffen School of Medicine at UCLA, Torrance and Los Angeles, CA, USA. nqureshi@mail.cho.org
authors.forename: Naveen
authors.initials: N
authors.lastname: Qureshi
authors.forename: James J
authors.initials: JJ
authors.lastname: Joyce
authors.forename: Ning
authors.initials: N
authors.lastname: Qi
authors.forename: Ruey-Kang
authors.initials: RK
authors.lastname: Chang
mesh_terms: 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*