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Updated: Jul 4, 2025
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No Increase in Masked Hypertension Prevalence in Children With Sickle Cell Disease in France
Plamen Bokov1, Berengere Koehl2,3, Chérine Benzouid4
1Paris University, AP-HP, Robert Debré Hospital, Department of Physiology, INSERM NeuroDiderot, Paris, France.
Insights
Children with sickle cell disease (SCD) show autonomic nervous system dysfunction, leading to increased cerebral blood flow velocity. This dysfunction is not linked to a higher prevalence of masked hypertension in pediatric SCD patients.
Area of Science:
- Pediatric Hematology
- Cardiovascular Physiology
- Neurology
Background:
- Sickle cell disease (SCD) is associated with a high prevalence of masked hypertension in children.
- Cerebral vasculopathy, detected by transcranial Doppler (TCD) ultrasound, is a known complication.
- Stroke screening using TCD is standard practice in pediatric SCD.
Purpose of the Study:
- To determine the prevalence of hypertension in children with SCD.
- To investigate the relationship between hypertension, cerebral vasculopathy (TCD velocity), and autonomic nervous system (ANS) function.
- To evaluate ANS indices in relation to TCD velocity in a subgroup of children.
Main Methods:
- Ambulatory blood pressure measurement (ABPM) and TCD velocity were assessed in children with SCD.
- Cardiac sympathovagal balance, baroreflex sensitivity, and pulse wave velocity were measured in a subset of participants.
- Heart rate variability analyses were employed to assess ANS function.
Main Results:
- Masked hypertension was found in 5% of children with SCD, with 10% having elevated blood pressure and 46% lacking a normal nocturnal BP dip.
- Increased TCD velocity correlated with reduced nocturnal systolic blood pressure dipping.
- In a subgroup, elevated TCD velocity was associated with parasympathetic withdrawal and baroreflex failure, alongside exaggerated orthostatic responses.
Conclusions:
- Autonomic nervous system dysfunction, specifically impaired parasympathetic modulation, contributes to increased TCD velocity in pediatric SCD.
- This ANS dysfunction is not associated with a higher prevalence of masked hypertension in this population.
- Findings highlight the complex interplay between ANS, cerebral hemodynamics, and blood pressure regulation in childhood SCD.
Background:
An important prevalence (32%-45%) of masked hypertension has been reported in children with sickle cell disease (SCD). Stroke screening is well established using transcranial Doppler (TCD) ultrasound. The objectives of our proof-of-concept study in childhood SCD were to evaluate the prevalence of hypertension and its relationships with cerebral vasculopathy (TCD velocity) and to further evaluate in a subgroup of children the correlations of cardiovascular autonomic nervous system indices with TCD velocity.
Methods:
Ambulatory blood pressure measurement (ABPM) and TCD velocity were obtained in children with SCD and in a restricted sample, cardiac sympathovagal balance using heart rate variability analyses, baroreflex sensitivity, and pulse wave velocity were measured.
Results:
In 41 children with SCD (median age 14.0 years, 19 girls, SS/Sβ + thalassemia/SC: 33/2/6), ABPM results showed masked hypertension in 2/41 (5%, 95% confidence interval, 0-11) children, consistent with the prevalence in the general pediatric population, elevated blood pressure (BP) in 4/41 (10%) children, and a lack of a normal nocturnal dip in 19/41 children (46%). Children with increased TCD velocity had lower nocturnal dipping of systolic BP. In the 10 participants with extensive cardiovascular assessment, increased TCD velocity was associated with parasympathetic withdrawal and baroreflex failure. Exaggerated orthostatic pressor response or orthostatic hypertension was observed in 7/10 children that was linked to parasympathetic withdrawal.
Conclusions:
Autonomic nervous system dysfunction, namely loss of parasympathetic modulation, of SCD contributes to increase TCD velocity but is not associated with an increased prevalence of masked hypertension.
Clinical Trials Registration:
NCT04911049.
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