Abstract - Increased Carotid Wall Stress in Vascular Ehlers-Danlos Syndrome

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Increased Carotid Wall Stress in Vascular Ehlers-Danlos Syndrome

Pierre Boutouyrie MD, PhD, Dominique P. Germain MD, PhD, Jean-Noël

Fiessinger MD, Brigitte Laloux PhD, Jérôme Perdu MD, and Stéphane t

MD, PhD*

From the Departments of Pharmacology and INSERM EMI 0107 (P.B., B.L., S.L.),

Clinical Genetics (D.P.G.), and Vascular Medicine (J.-N.F., J.P.), Hôpital

Européen s Pompidou, Paris, France.

* To whom correspondence should be addressed. E-mail:

stephane.laurent@....

Background--Vascular Ehlers-Danlos syndrome (vEDS), also known as EDS type

IV, an inherited disorder of connective tissue, results from mutations in

the gene encoding type III procollagen (COL3A1). Affected patients are at

risk for arterial dissection or rupture, the main cause of death. To

understand the pathogenesis of the vascular lesions, we used a biomechanical

approach and determined steady and pulsatile wall stress.

Methods and Results--Sixteen patients with vEDS and 16 age-, gender-, and

blood pressure-matched control subjects were included in this

cross-sectional noninvasive study. Circumferential wall stress was

determined under steady and pulsatile conditions at the site of an elastic

(common carotid) and a muscular (radial) artery from the measurements of

intima-media thickness and internal diameter with high-resolution

echo-tracking systems and either mean blood pressure or pulse pressure,

respectively. At the site of the carotid artery, steady circumferential wall

stress was 43% higher in vEDS patients than in control subjects (68.9±14.3

versus 48.2±12.1 kPa, P<0.001), and pulsatile circumferential wall stress

was 22% higher (28.2±7.7 versus 23.1±5.7 kPa, P<0.001). Carotid intima-media

thickness was 32% lower (408±56 versus 598±171 µm, P<0.001) in vEDS

patients, and internal diameter was not different between groups. Radial

artery parameters were not significantly different between groups.

Conclusions--In vEDS patients, an abnormally low intima-media thickness

generates a higher wall stress than in control subjects at the site of an

elastic artery, which may increase the risk of arterial dissection and

rupture.