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Ultraschall Med. 2018 Sep 21. doi: 10.1055/a-0599-0841. [Epub ahead of print]

Arterial Stiffening with Ultrafast Ultrasound Imaging Gives New Insight into Arterial Phenotype of Vascular Ehlers-Danlos Mouse Models.

Author information

1
Institut Langevin, INSERM U979, CNRS UMR 7587, ESPCI Paris, PSL Research University, Paris France.
2
USPC Sorbonne Paris Cité, Paris Descartes University, Paris, France.
3
INSERM UMR_S1140, Faculté de Pharmacie, Paris, France.
4
PARCC, INSERM U970, Paris, France.
5
Hôpital Européen Georges-Pompidou, Assistance Publique - Hôpitaux de Paris, France.

Abstract

in English, German

OBJECTIVE:

 Vascular Ehlers-Danlos syndrome (vEDS) is associated with arterial ruptures due to a mutant gene encoding collagen type III (Col-III). To better understand the role of Col-III, we aimed at evaluating aortic stiffness and dynamic stiffening in vEDS mouse models, with either a quantitative (col3KO mice) or a qualitative Col-III defect (col3KI mice).

MATERIALS AND METHODS:

 Abdominal aortic wall pulse wave velocities (PWV) in col3KO and col3KI mice were compared to their respective wild type (WT) littermates using a 15 MHz ultrafast ultrasonic transducer. A carotid catheter continuously monitored pressure changes due to phenylephrine injections. PWV1, generated at diastolic blood pressure (DBP), and PWV2, at systolic blood pressure (SBP) were recorded. Difference between PWV2 and PWV1 (Delta-PWV) normalized by the pulse pressure (PP), corresponding to the aortic stiffening over the cardiac cycle, were compared between mutant and WT mice, as well as the regression slope of PWV as a function of pressure.

RESULTS:

 Delta-PWV/PP was lower in col3KO (p = 0.033) and col3KI mice (p < 0.001) vs. WT-mice regardless of the pressure level. The slope of PWV1 with DBP increase showed a lower arterial stiffness in mutant mice vs. controls in both models. This difference was amplified when evaluating stiffness at systolic blood pressure levels with PWV2.

CONCLUSION:

 In both vEDS mouse models, aortic stiffening was reduced, mainly driven by a lower stiffness at systolic blood pressure. Defective Col-III may be responsible for this, as it is utilized when pressure rises. These pre-clinical data could explain vascular fragility observed in vEDS patients.

PMID:
30241104
DOI:
10.1055/a-0599-0841

Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

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