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Ventricular Structure and Function in Children With Sickle Cell Disease Using Conventional and Tissue Doppler Echocardiography

  • Ahmad Charaf Eddine
    Affiliations
    Division of Cardiology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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  • Ofelia Alvarez
    Affiliations
    Division of Hematology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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  • Steven E. Lipshultz
    Affiliations
    Division of Cardiology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA

    Division of Pediatric Clinical Research, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA

    Department of Epidemiology and Public Health, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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  • Richard Kardon
    Affiliations
    Division of Cardiology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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  • Kristopher Arheart
    Affiliations
    Division of Pediatric Clinical Research, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA

    Department of Epidemiology and Public Health, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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  • Sethuraman Swaminathan
    Correspondence
    Corresponding author: Tel: 305-585-6683; fax: 305-324-6012
    Affiliations
    Division of Cardiology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital at the University of Miami/Jackson Memorial Medical Center, Miami, Florida, USA
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Published:February 17, 2012DOI:https://doi.org/10.1016/j.amjcard.2012.01.001
      Conventional 2-dimensional, M-mode, and spectral Doppler echocardiographic techniques have documented abnormal ventricular function in adults with sickle cell disease (SCD), but assessments in children are conflicting. Tissue Doppler echocardiography (TDE) provides additional information about myocardial function. Two-dimensional, M-mode, tricuspid regurgitation jet velocity (TRJV) data, and tissue Doppler echocardiographically derived myocardial velocity measurements of left ventricular (LV) and right ventricular function were taken from children with SCD compared to those of similar healthy historical controls and correlated with clinical characteristics and hemoglobin levels. Compared to 55 controls, 54 children with SCD (mean age 14.2 years, range 6 to 21) had a larger left ventricle, greater LV mass, and higher LV fractional shortening; 30% had increased pulmonary artery pressure (TRJV ≥2.5 m/s). Conventional echocardiographic measurements of LV systolic function and spectral Doppler measurements of LV and right ventricular diastolic function were essentially normal, but TDE indicated that 31% of SCD children had evidence of LV diastolic dysfunction (peak early diastolic velocity of LV inflow Doppler/peak early diastolic velocity at lateral mitral valve annulus >8), a finding that correlated with lower hemoglobin levels. Although decreasing hemoglobin levels in children with SCD correlated with LV hypertrophy, LV dilation, and LV diastolic dysfunction, long-term transfusion or hydroxyurea therapy did not affect these measurements. In conclusion, 1/3 of children with SCD had tissue Doppler echocardiographic evidence of LV diastolic dysfunction, which was correlated with hemoglobin levels. Adding serial assessments of ventricular function with TDE to conventional echocardiography may detect early cardiac changes, especially in children with severe anemia.
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