The Journal of Pediatrics
Volume 149, Issue 1 , Pages 23-27, July 2006

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

  • Naveen Qureshi, MD

      Affiliations

    • Corresponding Author InformationReprint requests: Dr. Naveen Qureshi, Children’s Hospital and Research Center at Oakland, Department of Pediatric Hematology/Oncology, 757 Fifty-Second Street, 2nd Floor, Oakland, CA 94609.
    • ,
  • James J. Joyce, MD
    • ,
  • Ning Qi, MD
    • ,
  • Ruey-Kang Chang, MD, MPH

From the Department of Pediatrics, David Geffen School of Medicine at UCLA, Torrance and Los Angeles, CA; Department of Pediatric Hematology/Oncology, Children’s Hospital and Research Center at Oakland, Oakland, CA; and Division of Pediatric Cardiology, University of Florida Health Science Center, Jacksonville, FL.

Received 4 April 2005; received in revised form 29 November 2005; accepted 23 December 2005.

Objective

To assess the effects of sickle cell anemia (SCA) on the right ventricle (RV).

Study design

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 (ETc), 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 cm2/m2 vs 13.2 ± 2.1 cm2/m2; P < .01) and free-wall mass index (33.2 ± 4.4 g/m2 vs 23.9 ± 4.3 g/m2; 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.

Abbreviations:  BSA, Body surface area , ETc, Ejection time corrected for heart rate , RV, Right ventricle , SCA, Sickle cell anemia , TR, Tricuspid regurgitation

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PII: S0022-3476(05)01241-2

doi:10.1016/j.jpeds.2005.12.055

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The Journal of Pediatrics
Volume 149, Issue 1 , Pages 23-27, July 2006

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