title: Arterial stiffness and wave reflections in patients with sickle cell disease.
abstract: We tested the hypothesis that lower blood pressure and increased vasodilatation reported in sickle cell disease (SCD) patients with hemoglobin SS genotype (SS) are translated by lower arterial stiffness determined by pulse wave velocity (PWV) and wave reflections assessed by augmentation index (AI). We enrolled 20 SS (8 females; 12 male) patients closely matched for age, gender, height, and body mass index to 20 subjects with hemoglobin AA genotype (AA). Carotid-femoral PWV (PWV(CF)) and carotid-radial PWV (PWV(CR)) were recorded with the Complior device. Aortic AI was derived from pressure wave analysis (SphygmocoR). PWV(CF) and PWV(CR) were lower in SS than in AA (4.5+/-0.7 m/s versus 6.9+/-0.9 m/s, P<0.0001 and 6.6+/-1.2 m/s versus 9.5+/-1.4 m/s, P<0.0001, respectively). AI was lower in SS than in AA (2+/-14% versus 11+/-8%, P=0.02). Multivariate analysis revealed that both PWV(CF) and PWV(CR) were negatively associated with hemoglobin SS type and positively related to mean arterial pressure (MAP), whereas AI was positively associated with MAP and total cholesterol (all P<0.0001). Multivariate analysis restricted to SS indicated a positive association between PWV(CF) and PWV(CR) with age but a negative association with MAP (R2=0.57 and 0.51, respectively, both P<0.001), whereas MAP and heart rate were independently associated with AI (R2=0.65, P<0.001). This study provides the first evidence that SCD is associated with both lower arterial stiffness and wave reflections. SS patients have a paradoxical negative association between PWV and MAP, suggesting that low MAP does not protect them against arterial stiffness impairment.
authors.affiliation: Hypertension Clinic, Department of Cardiology, Erasme Hospital, 808 Lennik Road, 1070 Brussels, Belgium. pvandebo@ulb.ac.be
authors.forename: Daniel
authors.initials: D
authors.lastname: Lemogoum
authors.forename: Luc
authors.initials: L
authors.lastname: Van Bortel
authors.forename: Boutaina
authors.initials: B
authors.lastname: Najem
authors.forename: Anasthase
authors.initials: A
authors.lastname: Dzudie
authors.forename: Charles
authors.initials: C
authors.lastname: Teutcha
authors.forename: Ernest
authors.initials: E
authors.lastname: Madu
authors.forename: Marc
authors.initials: M
authors.lastname: Leeman
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Degaute
authors.forename: Philippe
authors.initials: P
authors.lastname: van de Borne
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000503:physiopathology*; D001158:Arteries / Q000502:physiology*; D001783:Blood Flow Velocity; D001794:Blood Pressure / Q000502:physiology*; D005260:Female; D006339:Heart Rate; D006441:Hemoglobin A; D006451:Hemoglobin, Sickle; D006801:Humans; D008297:Male; D012044:Regression Analysis; D014664:Vasodilation / Q000502:physiology*
chemical_list: D006451:Hemoglobin, Sickle; C028672:hemoglobin AA; D006441:Hemoglobin A