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.
Abstract:
Diastolic dysfunction has been shown to occur earlier than systolic dysfunction in iron overload states in adult patients with sickle cell disease (SCD). Tissue Doppler imaging (TDI)-derived E/E' has emerged as a noninvasive marker of diastolic function. We sought to determine diastolic function in children with SCD and study its relation with iron overload. A retrospective review of medical records of 225 pediatric patients with SCD who received an echocardiogram between January 2008 and December 2012 was performed. Echocardiographic measures including M-mode, spectral Doppler, and TDI-derived E/E' were compared with previously published data in healthy children. The left ventricular end-diastolic and end-systolic dimensions were significantly higher in SCD (P<0.0001) and the shortening fraction was similar (P=0.66). E/E' ratio was significantly higher in SCD at the mitral annulus, septum, and tricuspid annulus. In 54% of subjects, the septal E/E' was >8, indicating elevated left ventricular filling pressure. However, there was no significant correlation between ferritin level and E/E' ratios. Pediatric patients with SCD have a high prevalence of elevated estimated left ventricular filling pressure, but this does not correlate with ferritin levels.
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