Dyssynchronous systolic expansion of carotid artery in patients with marfan syndrome

Abstract

Background: Marfan syndrome is a multisystemic connective tissue disorder associated with a mutation affecting fibrillin-1, the main component of microfibrils. Fibrillin-1 gene mutations may affect the carotid arterial wall. The aim of this study was to investigate carotid arterial mechanics using Velocity Vector Imaging (VVI) in patients with Marfan syndrome.

Methods: Forty-five patients (26 men; mean age, 39 ± 10 years) with Marfan syndrome who fulfilled the Ghent criteria and 45 gender-matched and age-matched healthy volunteers were evaluated. Transverse images of right common carotid artery proximal to the bifurcation were obtained for each subject and divided into six segments. The peak radial velocity, circumferential strain, and strain rate of the six segments were analyzed using VVI. The time to peak radial velocity (T(s)), peak circumferential strain (T(st)), and peak strain rate (T(sr)) of the six segments were calculated. Intima-media thickness was measured for each subject.

Results: The average diameter of the common carotid artery in patients with Marfan syndrome was significantly larger than that of controls. Carotid compliance coefficients and distensibility coefficients as assessed by B-mode echocardiographic images were comparable between the two groups. In VVI analyses, averages and standard deviations of peak radial velocities, circumferential strain, and strain rates were not significantly different between the two groups. However, T(s), T(st), and T(sr) were more delayed (P < .01), and the standard deviations of T(s), T(st), and T(sr) were significantly larger in patients with Marfan syndrome (P = .01, P < .01, and P < .01, respectively), suggesting delayed and dyssynchronous arterial expansion during systole. The presence of Marfan syndrome was independently and significantly related to increased standard deviations of T(st) (β = 0.33, P < .01) and T(sr) (β = 0.44, P < .01), even after adjusting for age in multiple regression analysis.

Conclusion: In patients with Marfan syndrome, carotid arteries assessed with VVI exhibited delayed, dyssynchronous arterial expansion during systole compared with healthy controls. Arterial assessment using VVI may be useful for noninvasively quantifying vascular alterations associated with Marfan syndrome.