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Ventricular global longitudinal strain is altered in children with sickle cell disease

Nicholas S Whipple1, Ronak J Naik2,3, Guolian Kang4

  • 1Department of Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.

British Journal of Haematology
|November 20, 2018
PubMed

Insights

Right ventricular global longitudinal strain (RVGLS) is elevated in children with sickle cell disease (SCD), indicating early cardiac changes. This RVGLS metric may serve as a key indicator of altered heart function in pediatric SCD patients.

Area of Science:

  • Cardiology
  • Pediatrics
  • Hematology

Background:

  • Cardiac disease is the leading cause of mortality in sickle cell disease (SCD).
  • Global longitudinal strain (GLS) of the right and left ventricles (RVGLS, LVGLS) are sensitive indicators of systolic dysfunction.
  • Limited research exists on RVGLS and LVGLS in pediatric SCD populations.

Purpose of the Study:

  • To investigate RVGLS and LVGLS in children with SCD.
  • To compare GLS parameters between SCD patients and healthy controls.
  • To explore associations between GLS, clinical parameters, and markers of hemolysis and inflammation.

Main Methods:

  • Echocardiography was used to measure RVGLS and LVGLS in 143 children with SCD (HbSS or HbSβ0-thalassemia) and 71 age- and sex-matched controls.
  • Measurements were compared with conventional echocardiographic parameters, hemolysis markers, and inflammation markers.
  • Statistical analyses, including correlation and regression, were performed to assess relationships.

Main Results:

  • RVGLS was significantly higher in children with SCD compared to controls (P=0.005).
  • LVGLS did not differ between groups.
  • RVGLS decreased with increasing age in SCD patients (ρ=-0.338, P<0.001) and was associated with increased left atrial volume and cardiac output.

Conclusions:

  • Elevated RVGLS in pediatric SCD patients may represent an early compensatory mechanism in response to myocardial stress and increased cardiac output.
  • Global longitudinal strain, particularly RVGLS, shows potential as an early biomarker for myocardial dysfunction in children with SCD.
  • Further research is warranted to elucidate the long-term implications of these findings.

Keywords:
cardiomyopathyglobal longitudinal strainheartsickle cell diseasespeckle-trackingstrain

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