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  • Title: Relation between age, arterial distensibility, and aortic dilatation in the Marfan syndrome.
    Author: Jeremy RW, Huang H, Hwa J, McCarron H, Hughes CF, Richards JG.
    Journal: Am J Cardiol; 1994 Aug 15; 74(4):369-73. PubMed ID: 8059700.
    Abstract:
    This study examined the relations between age, arterial distensibility, and systemic hemodynamics in patients with the Marfan syndrome. The study group included 170 patients referred to a specialist clinic, of whom 55 (age 26 +/- 12 years) were diagnosed as having Marfan syndrome. The remaining 115 patients (age 25 +/- 14 years) formed a control group. Each patient underwent echocardiographic examination, with measurement of ascending aorta diameter at end-diastole and end-systole, and aortic flow velocities. The elastic properties of the aorta were indexed by calculation of aortic distensibility, wall stiffness, and systemic pulse wave velocity. Mean end-diastolic aortic diameter in the Marfan group (38 +/- 9 mm) was greater than that in the controls (26 +/- 4 mm, p < 0.01). Resting heart rate and aortic flow velocities were similar in the 2 groups, but systemic arterial pulse pressure was greater in the Marfan group (50 +/- 12 mm Hg) than in the controls (41 +/- 8 mm Hg, p < 0.01). Aortic diameter increased with age in both groups, but at all ages the Marfan group exhibited greater aortic diameters (p < 0.05). Aortic distensibility was less in the Marfan group (2.6 +/- 1.3 cm2.dynes-1 x10(-6)) than in the controls (6.2 +/- 2.1 cm2.dynes-1 x 10(-6), p < 0.01), and the aortic wall stiffness index was greater in the Marfan group (7.9 +/- 3.4) than in the controls (2.8 +/- 0.6, p < 0.01). Aortic wall stiffness increased with age and aortic diameter, but at all ages the Marfan group exhibited a stiffer aorta for a given diameter than did the controls.(ABSTRACT TRUNCATED AT 250 WORDS)
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