Tissue Doppler Imaging-derived Diastolic Function Assessment in Children With Sickle Cell Disease and Its Relation

Michelle Olson1, Camden Hebson, Alexandra Ehrlich

  • 1*Department of Pediatrics †Sibley Heart Center Cardiology ‡Division of Hematology-Oncology, Children's Healthcare of Atlanta and Emory University School of Medicine, Atlanta, GA.

Insights

Pediatric patients with sickle cell disease (SCD) often show early signs of diastolic dysfunction, indicated by elevated left ventricular filling pressures. However, this dysfunction did not correlate with iron overload markers like ferritin levels in this study.

Area of Science:

  • Cardiology
  • Hematology
  • Pediatrics

Background:

  • Diastolic dysfunction can precede systolic dysfunction in sickle cell disease (SCD) patients with iron overload.
  • Tissue Doppler imaging (TDI)-derived E/E' is a noninvasive marker for diastolic function.

Purpose of the Study:

  • To assess diastolic function in pediatric patients with SCD.
  • To investigate the relationship between diastolic function and iron overload in this population.

Main Methods:

  • Retrospective review of 225 pediatric SCD patients' echocardiograms (2008-2012).
  • Analysis of M-mode, spectral Doppler, and TDI-derived E/E' ratios.
  • Comparison with healthy children's data.

Main Results:

  • Pediatric SCD patients had significantly higher left ventricular dimensions (P<0.0001) but similar shortening fraction (P=0.66) compared to controls.
  • Elevated E/E' ratios (indicating increased left ventricular filling pressure) were observed in SCD patients at the mitral annulus, septum, and tricuspid annulus.
  • 54% of subjects had septal E/E' >8, suggesting elevated filling pressures.
  • No significant correlation was found between ferritin levels and E/E' ratios.

Conclusions:

  • Pediatric patients with SCD exhibit a high prevalence of elevated left ventricular filling pressures.
  • These elevated filling pressures in pediatric SCD patients do not correlate with ferritin levels, suggesting other factors may contribute to diastolic dysfunction.