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P1.10 Longitudinal Changes in Geometric and Functional Arterial Properties in Vascular Ehlers-Danlos Syndrome with Celiprolol

Abstract

Objective

Vascular Ehlers-Danlos (vEDS) syndrome is a rare disease (1/100,000), due to mutations in the collagen type III (COL3A1). vEDS is characterized by early spontaneous arterial rupture or dissection. Celiprolol, a beta1 antagonist beta2 partial agonist conferred protection against CV events with paradoxical stiffening effects (Ong et al, Lancet 2010). Our aim was evaluate celiprolol effect on arterial properties during a long term longitudinal follow-up of a large population of patients.

Methods

63 patients (age 35±10, 57% females) having at least 2 visit were followed 5 years during 6±3 visits. Carotid internal diastolic diameter (Di), intima-media thickness (IMT), arterial wall cross-sectional (WCSA), circumferential wall stress, distensibility and Young’s elastic modulus (Einc) were measured. The evolution over time in response to celiprolol was studied using mixed models.

Results

46 patients were exposed to celiprolol. SBP increased with time under celiprolol (0.79 mmHg/y, p<0.001), so did central SBP (0.89 mmHg/y; p = 0.002) and central PP (1.24 mmHg/y, p<0.001), without changed heart rate. Di and IMT increased (+36μm/y, p<0.001 and +4.4 μm/y, p<0.001, respectively). Einc increased (29.92 kPa/y, p<0.001) and distensibility decreased (−0.003 kPa-1/y, p<0.001). In unexposed patients (n = 17), brachial BP did not change significantly, whereas changes in arterial wall properties were similar to those exposed to celiprolol.

Conclusion

The effect of time on large arteries properties seems similar whether patients are treated with celiprolol or not. Changes might thus be due to aging process rather that to pharmacologically induced changes.

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Hoang, P., Laurent, S., Franck, M. et al. P1.10 Longitudinal Changes in Geometric and Functional Arterial Properties in Vascular Ehlers-Danlos Syndrome with Celiprolol. Artery Res 8, 131 (2014). https://doi.org/10.1016/j.artres.2014.09.091

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  • DOI: https://doi.org/10.1016/j.artres.2014.09.091