Objective: Vascular Ehlers–Danlos syndrome (vEDS) is a rare connective tissue disorder caused by pathogenic variants in COL3A1, characterized by arterial wall fragility, reduced intima–media thickness (IMT), and increased circumferential wall stress. While celiprolol reduces major arterial events, its long-term impact on arterial biomechanics and vascular aging remains incompletely defined. Therefore, this study aimed to characterize arterial stiffness, central hemodynamics, and vascular remodeling in vEDS patients receiving long-term celiprolol therapy compared to healthy controls. Design and method: Monocentric cross-sectional study including 10 patients with molecularly confirmed vEDS receiving chronic celiprolol therapy and 10 age- and sex-matched healthy controls. All participants underwent assessment of carotid–femoral pulse wave velocity (cfPWV), central blood pressure and wave reflection indices, and carotid echo-tracking for structural and local biomechanical parameters. Circumferential wall stress was calculated using Laplace's law. Group comparisons were performed using unadjusted and adjusted analyses, including covariance models, multivariable regression analyses, and exploratory subgroup evaluations. Results: cfPWV was significantly higher in vEDS patients than in controls (9.33 ± 1.18 vs 7.56 ± 1.22 m/s; p=0.004), with a large effect size (Cohen's d=1.47), and remained independently associated with vEDS after adjustment for central mean blood pressure and age (beta=1.83 m/s, 95% confidence interval 0.71–2.95; p=0.003). Central hemodynamic parameters and wave reflection indices were comparable between groups. Carotid IMT (0.439 ± 0.067 vs 0.488 ± 0.134 mm) was numerically lower in vEDS patients, with moderate effect sizes, while local biomechanical indices did not differ significantly. Mean circumferential wall stress was numerically higher in vEDS patients (82.3 ± 22.1 vs 76.6 ± 13.9 kPa) and showed a strong inverse association with IMT across the cohort (rho=–0.736; p<0.001), preserved in controls but attenuated in vEDS. Exploratory analyses revealed reduced IMT in vEDS patients with prior vascular events (0.42 ± 0.06 vs 0.49 ± 0.07 mm), with a large effect size (Cohen's d=–1.08). Conclusions: In patients with vEDS receiving celiprolol therapy, increased aortic stiffness was observed despite relatively preserved carotid structure and central hemodynamics, suggesting heterogeneous vascular remodeling consistent with an early vascular aging phenotype.
Buso et al. (Fri,) studied this question.