Dynamic Cerebral Autoregulation in Homozygous Sickle Cell Disease
Open accessAbstract
Background and Purpose— Sickle cell disease (SCD) is associated with cerebral hyperperfusion and an increased risk of stroke. Also, both recurrent microvascular obstruction and chronic hemolysis affect endothelial function, potentially interfering with systemic and cerebral blood flow control. We addressed the question whether cerebrovascular control in patients with SCD is affected and related to hemolysis. Methods— Systemic and cerebrovascular control were studied in 18 patients with SCD and 10 healthy subjects. Dynamic cerebral autoregulation was evaluated by transfer function analysis assessing the relationship between mean cerebral blood flow velocity and mean arterial pressure. Results— Normal baroreflex sensitivity and postural cardiovascular reflex responses indicated integrity of systemic cardiovascular control. In the low- (0.07 to 0.15 Hz) frequency region, mean arterial pressure variability was comparable for both groups, but a larger mean cerebral blood flow velocity variability in SCD (6.1 [4.6 to 7.0] versus 4.2 [2.6 to 5.2] [cm·s −1 ] 2 ·Hz −1 ; P <0.05) indicated a reduced capacity to buffer the transfer of blood pressure surges to the cerebral tissue. Impairment of dynamic cerebrovascular control was confirmed by a reduced mean arterial pressure-to-mean cerebral blood flow velocity transfer function phase lead in SCD versus healthy subjects (32±17° versus 50±19°, P <0.05) that was unrelated to the severity of hemolysis. Conclusions— In patients with SCD, dynamic cerebral autoregulation is impaired but appears unrelated to hemolysis.
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Cerebral autoregulatory capacity is impaired in sickle cell disease.
“the cerebral autoregulatory capacity, i.e., the capacity to adjust vessel volume in response to carbon monoxide challenge or systemic blood pressure changes, is impaired in SCD (137, 138).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [137]
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Pathophysiology of Sickle Cell Disease
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- Authors
- Kim, Y.-S., Nur, E., van Beers, E. J., Truijen, J., Davis, S. C. A. T., Biemond, B. J., & van Lieshout, J. J.
- Affiliations
- Slotervaartziekenhuis (NL)
- From the Department of Internal Medicine (Y.-S.K., E.J.v.B., J.T., B.J.B., J.J.v.L.) and the Laboratory for Clinical Cardiovascular Physiology (Y.-S.K., J.T., S.C.A.T.D., J.J.v.L.), AMC Center for Heart Failure Research, Academic Medical Center, Amsterdam, The Netherlands; the Department of Internal Medicine (E.N.), Slotervaart Hospital, Amsterdam, The Netherlands; The CURAMA study group (E.N., E.J.v.B., B.J.B.); and the Department of Hematology (E.N., E.J.v.B., B.J.B.), Academic Medical Center,...
- Venue
- Stroke
- Published
- 2009-03
- Volume
- 40
- Issue
- 3
- Pages
- 808-814
- ISSN
- 0039-2499 (ISSN-L)
- 1524-4628
- OpenAlex
- W1983003198
- Cited by
- 34
- References
- 48
- Citations by year
- 2026: 2 · 2025: 1 · 2024: 3 · 2021: 1 · 2020: 1 · 2019: 2 · 2018: 4 · 2017: 3 · 2016: 4 · 2015: 1 · 2014: 3 · 2013: 1 · 2012: 3
- FWCI
- 1.26
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Stroke (publishedVersion)
- PubMed (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Blood properties and coagulation
- Keywords
- sickle cell disease, dynamic cerebral autoregulation, cerebral blood flow velocity, hemolysis, baroreflex sensitivity, cerebrovascular control, stroke risk, cerebral hyperperfusion
- MeSH terms
- Adult, Anemia, Sickle Cell, Blood Cell Count, Blood Pressure, Cerebrovascular Circulation, Female, Heart Rate, Hemodynamics, Hemolysis, Homeostasis, Humans, Male, Ultrasonography, Doppler, Transcranial
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