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Factors associated with left ventricular hypertrophy in children with sickle cell disease: results from the DISPLACE

Najibah A Galadanci1, Walter Johnson2, April Carson3

  • 1Division of Hematology and Oncology, UAB School of Medicine, University of Alabama at Birmingham, Birmingham.

Haematologica
|April 14, 2022
PubMed

Insights

Left ventricular hypertrophy (LVH) is common in children with sickle cell disease (SCD). Higher age and lower hemoglobin increase LVH risk, while hydroxyurea therapy is associated with increased odds of LVH.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Cardiopulmonary complications are major causes of illness and death in sickle cell disease (SCD).
  • Left ventricular hypertrophy (LVH) is a significant cardiac complication in SCD patients.
  • Understanding LVH's relationship with hemolysis markers and SCD therapies is crucial for patient management.

Purpose of the Study:

  • To investigate the association between left ventricular hypertrophy (LVH) and laboratory markers of hemolysis in children with SCD.
  • To determine the relationship between LVH and SCD-specific treatments, including hydroxyurea and chronic red cell transfusions.
  • To identify risk factors for early development of LVH in pediatric SCD patients.

Main Methods:

  • Utilized data from the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study cohort.
  • Defined LVH using left ventricular mass indexed to body surface area (LVMI >103.0 g/m2 for males, >84.2 g/m2 for females).
  • Performed multivariable analysis on 1,409 children with SCD to assess factors associated with LVH.

Main Results:

  • 20.3% of pediatric SCD patients in the cohort exhibited LVH.
  • Lower baseline hemoglobin levels (OR: 0.71) and male sex (OR: 0.59) were associated with decreased odds of LVH.
  • Increasing age (OR: 1.07) and hydroxyurea therapy (OR: 1.83) were associated with increased odds of LVH.

Conclusions:

  • LVH is prevalent in children with SCD and its risk escalates with age and diminished hemoglobin levels.
  • The association between hydroxyurea use and LVH suggests a potential link between this therapy and cardiovascular remodeling in pediatric SCD.
  • Early identification and management of LVH risk factors are essential in children with SCD.

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