Arterial Stiffness and Wave Reflections in Patients With Sickle Cell Disease

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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 P 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 CF and PWV CR with age but a negative association with MAP ( R 2 =0.57 and 0.51, respectively, both P R 2 =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.