American Journal of Cardiology
Volume 104, Issue 5 , Pages 713-720, 1 September 2009

Prospective Echocardiography Assessment of Pulmonary Hypertension and Its Potential Etiologies in Children With Sickle Cell Disease†

  • Niti Dham, MD

      Affiliations

    • Department of Cardiology, Children's National Medical Center, Washington, District of Columbia
    • ,
  • Gregory Ensing, MD

      Affiliations

    • Pediatric Cardiology Division, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan
    • ,
  • Caterina Minniti, MD

      Affiliations

    • Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland
    • ,
  • Andrew Campbell, MD

      Affiliations

    • Hematology Division, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan
    • ,
  • Manuel Arteta, MD

      Affiliations

    • Pulmonary Division, Department of Pediatrics, University of Michigan, Ann Arbor, Michigan
    • ,
  • Sohail Rana, MD

      Affiliations

    • Department of Pediatrics and Child Health, Howard University, Washington, District of Columbia
    • ,
  • Deepika Darbari, MD

      Affiliations

    • Department of Hematology, Children's National Medical Center, Washington, District of Columbia
    • ,
  • Mehdi Nouraie, MD, PhD

      Affiliations

    • Center for Sickle Cell Disease, Howard University College of Medicine, Washington, District of Columbia
    • ,
  • Onyinye Onyekwere, MD

      Affiliations

    • Center for Sickle Cell Disease, Howard University College of Medicine, Washington, District of Columbia
    • ,
  • Malgorzata Lasota, RDCS

      Affiliations

    • Department of Cardiology, Children's National Medical Center, Washington, District of Columbia
    • ,
  • Gregory J. Kato, MD

      Affiliations

    • Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland
    • ,
  • Mark T. Gladwin, MD

      Affiliations

    • Department of Pulmonary, Allergy and Critical Care Medicine and Hemostasis and Vascular Biology Research Institute, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
    • ,
  • Oswaldo Castro, MD

      Affiliations

    • Center for Sickle Cell Disease, Howard University College of Medicine, Washington, District of Columbia
    • ,
  • Victor Gordeuk, MD

      Affiliations

    • Center for Sickle Cell Disease, Howard University College of Medicine, Washington, District of Columbia
    • ,
  • Craig Sable, MD

      Affiliations

    • Department of Cardiology, Children's National Medical Center, Washington, District of Columbia
    • Corresponding Author InformationCorresponding author: Tel: 202-476-2020; fax: 202-476-5700

Received 17 February 2009; received in revised form 26 April 2009; accepted 26 April 2009. published online 26 June 2009.

Pulmonary hypertension (PH) is associated with adverse outcomes in adults with sickle-cell disease (SCD), but its importance in children is less clear. The aim of this study was to define the incidence and causes of PH in pediatric patients with SCD. Children with SCD (n = 310) and matched controls (n = 54) were prospectively enrolled under basal conditions. Participants underwent echocardiography, pulse oximetry, 6-minute walk tests, and hematologic testing. Echocardiographic measures were compared between patients with SCD and control subjects before and after adjusting for hemoglobin. Correlations of echocardiographic and clinical parameters were determined. Tricuspid regurgitation velocity (TRV) was elevated compared to controls (2.28 vs 2.10 m/s, p <0.0001). Increased TRV was associated with left ventricular diastolic diameter, hemoglobin, and estimated left atrial pressure. TRV remained elevated when controlling for left ventricular diameter and left atrial pressure. Echocardiographically derived pulmonary resistance was not significantly different between patients with SCD and controls, although it was elevated in the SCD subgroup with elevated TRV. When controlling for hemoglobin, TRV was no longer statistically different, but pulmonary insufficiency velocity, septal wall thickness, and estimated pulmonary resistance were statistically higher. TRV, pulmonary insufficiency end-diastolic velocity, and markers of increased cardiac output were correlated with indicators of adverse functional status, including history of acute chest syndrome, stroke, transfusions, and 6-minute walk distance. In conclusion, children with SCD had mildly increased TRV that was correlated with increased cardiac output and left ventricular filling pressures. Hemoglobin-adjusted analysis also suggested a contribution of primary vascular changes.

 
  • † Conflicts of interest: Dr. Gordeuk has received grants from BioMarin Pharmaceutical Inc., Novato, California, and Actelion Pharmaceuticals Ltd., Allschwil, Switzerland, and is a consultant for Ikaria Holdings, Clinton, New Jersey.

 This study was supported in part by Grants 2 R25 HL003679-08 and 1 R01 HL079912-02 from the National Heart, Lung, and Blood Institute, Bethesda, Maryland; by Howard University General Clinical Research Center Grant 2MOI RR10284-10 from the National Center for Research Resources, National Institutes of Health, Bethesda, Maryland; and by the Intramural Research Program of the National Institutes of Health.

PII: S0002-9149(09)01002-9

doi:10.1016/j.amjcard.2009.04.034

American Journal of Cardiology
Volume 104, Issue 5 , Pages 713-720, 1 September 2009

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