From the Department of Clinical and Experimental Medicine, University of Pisa, Italy (R.M.B.).
INSERM U970, Paris Cardiovascular Research Center (PARCC), France (R.M.B., L.M., X.J., S.L., P.B.).
Hypertension. 2019 Feb;73(2):371-378. doi: 10.1161/HYPERTENSIONAHA.118.12189.
Arterial fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease, whose pathophysiology is still unknown. We performed deep image-based vascular phenotyping of nonaffected arteries to look for systemic vascular alterations in fibromuscular dysplasia. This single center cross-sectional study included 50 patients with multifocal renal fibromuscular dysplasia, 50 hypertensive patients, and 50 healthy controls, matched for age, sex, and ethnicity; hypertensive patients were matched also for blood pressure. Brachial artery endothelium-dependent and endothelium-independent dilation were studied by echotracking. Aortic stiffness was assessed by carotid-to-femoral pulse wave velocity. We quantified the presence of supernumerary acoustic interfaces within the common carotid wall by the triple signal (TS) score. We plotted the Young incremental elastic modulus/stress curves for common carotid artery, derived from echotracking and tonometry. Patients with fibromuscular dysplasia had impaired endothelium-independent dilation (adjusted P=0.002), smaller brachial artery diameter but comparable endothelium-dependent dilation and aortic stiffness. The prevalence of TS score >6 was 56%, 40%, 24% in patients with fibromuscular dysplasia, hypertensives, and controls, respectively ( P=0.005). Fibromuscular dysplasia remained significantly associated with TS in the multiple regression model ( P=0.022). Impaired endothelium-dependent dilation was present only in patients with fibromuscular dysplasia, TS score >6 ( P=0.047). Incremental elastic modulus was higher for a given wall stress (80 kPa) in the presence of a TS score >6, especially in fibromuscular dysplasia. In conclusion, nonclinically affected large- and medium-sized arteries in patients with multifocal renal fibromuscular dysplasia exhibit a cluster of diffuse alterations in smooth muscle cell function, arterial geometry, wall characteristics, and mechanical properties. Clinical Trial Registration URL: http://www.clinicaltrials.gov . Unique identifier: NCT01935752.
动脉纤维肌性发育不良是一种非动脉粥样硬化性、非炎症性血管疾病，其病理生理学尚不清楚。我们对无病变动脉进行了深入的基于影像的血管表型分析，以寻找纤维肌性发育不良中的系统性血管改变。这项单中心横断面研究纳入了 50 例多发性肾纤维肌性发育不良患者、50 例高血压患者和 50 例健康对照者，匹配年龄、性别和种族；高血压患者还按血压进行匹配。通过超声心动图追踪研究肱动脉内皮依赖性和非依赖性扩张。通过颈股脉搏波速度评估主动脉僵硬度。我们通过三重信号 (TS) 评分量化颈总动脉壁内多余的声界面数量。我们根据超声心动图和张力测定法绘制颈总动脉的杨氏增量弹性模量/应力曲线。纤维肌性发育不良患者的非内皮依赖性扩张受损（调整后 P=0.002），肱动脉直径较小，但内皮依赖性扩张和主动脉僵硬度相当。纤维肌性发育不良患者、高血压患者和对照组的 TS 评分>6 的患病率分别为 56%、40%和 24%（ P=0.005）。在多元回归模型中，纤维肌性发育不良与 TS 仍显著相关（ P=0.022）。只有在纤维肌性发育不良患者中，TS 评分>6 时才存在内皮依赖性扩张受损（ P=0.047）。在存在 TS 评分>6 的情况下，对于给定的壁应力（80 kPa），增量弹性模量更高，尤其是在纤维肌性发育不良患者中。总之，多发性肾纤维肌性发育不良患者无临床症状的大中动脉表现出平滑肌细胞功能、动脉几何形状、壁特征和力学特性的弥漫性改变簇。临床试验注册网址：http://www.clinicaltrials.gov. 独特标识符：NCT01935752。
