title: Relation between age, arterial distensibility, and aortic dilatation in the Marfan syndrome.
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)
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, New South Wales, Australia.
authors.forename: R W
authors.initials: RW
authors.lastname: Jeremy
authors.forename: H
authors.initials: H
authors.lastname: Huang
authors.forename: J
authors.initials: J
authors.lastname: Hwa
authors.forename: H
authors.initials: H
authors.lastname: McCarron
authors.forename: C F
authors.initials: CF
authors.lastname: Hughes
authors.forename: J G
authors.initials: JG
authors.lastname: Richards
mesh_terms: D000328:Adult; D000367:Age Factors; D001011:Aorta / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D004108:Dilatation, Pathologic / Q000000981:diagnostic imaging / Q000473:pathology; D004452:Echocardiography; D004548:Elasticity; D005260:Female; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000503:physiopathology*; D014655:Vascular Resistance / Q000502:physiology*