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Updated: Jun 14, 2026

Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
Published on: November 5, 2019
Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and
Mark C Johnson1, Fenella J Kirkham, Susan Redline
1Department of Pediatrics, Washington University School of Medicine, 660 S. Euclid Avenue, St Louis, MO 63110, USA.
Insights
Low oxygen saturation during sleep and waking is linked to left ventricular abnormalities in children with sickle cell disease (SCD). This finding highlights the impact of breathing issues on cardiac health in pediatric SCD patients.
Area of Science:
- Cardiology
- Pediatrics
- Sleep Medicine
- Hematology
Background:
- Sickle cell disease (SCD) is associated with premature death and cardiac abnormalities.
- The underlying mechanisms connecting SCD to cardiac issues, particularly in children, remain unclear.
- Sleep-disordered breathing is a potential, yet understudied, contributor to cardiac dysfunction in pediatric SCD.
Purpose of the Study:
- To investigate the association between sleep-disordered breathing and cardiac abnormalities in children with SCD.
- To test the hypothesis that reduced oxygen saturation during sleep and waking contributes to cardiac dysfunction in this population.
Main Methods:
- An observational study involving 44 children with SCD (mean age 10.1 years).
- Evaluations included standard and tissue Doppler echocardiography, overnight polysomnography, and measurement of average asleep and waking oxygen saturation.
- Data were collected within a 7-day period for each participant.
Main Results:
- Eccentric left ventricular (LV) hypertrophy was identified in 46% of the children.
- Lower average asleep and waking oxygen saturation were significantly associated with increased LV mass index.
- LV diastolic dysfunction was also correlated with reduced oxygen saturation levels.
Conclusions:
- Low oxygen saturation during both sleep and waking is associated with left ventricular abnormalities in children with SCD.
- These findings suggest that sleep-disordered breathing may play a role in the cardiac complications observed in pediatric SCD.
- Further research is warranted to explore therapeutic interventions targeting sleep-disordered breathing in this vulnerable population.
Abstract:
Premature death and cardiac abnormalities are described in individuals with sickle cell disease (SCD), but the mechanisms are not well characterized. We tested the hypothesis that cardiac abnormalities in children with SCD are related to sleep-disordered breathing. We enrolled 44 children with SCD (mean age, 10.1 years; range, 4-18 years) in an observational study. Standard and tissue Doppler echocardiography, waking oxygen saturation averaged over 5 minutes, and overnight polysomnography were obtained in participants, each within 7 days. Eccentric left ventricular (LV) hypertrophy was present in 46% of our cohort. After multivariable adjustment, LV mass index was inversely related to average asleep and waking oxygen saturation. For every 1% drop in the average asleep oxygen saturation, there was a 2.1 g/m(2.7) increase in LV mass index. LV diastolic dysfunction, as measured by the E/E' ratio, was present in our subjects and was also associated with low oxygen saturation (sleep or waking). Elevated tricuspid regurgitant velocity (> or = 2.5 m/sec), a measure of pulmonary hypertension, was not predicted by either oxygen saturation or sleep variables with multivariable logistic regression analysis. These data provide evidence that low asleep and waking oxygen saturations are associated with LV abnormalities in children with SCD.
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