title: Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.
abstract: Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA). The mean value of flow-mediated dilation was 6.5 ± 2.4 %. Flow-mediated dilation had a negative correlation with the diameter of the ascending thoracic aorta (AscAd)/BSA (R = -0.39, p = 0.020) and multivariate analysis revealed that flow-mediated dilation was an independent factor predicting AscAd/BSA, whereas other segments of the aorta had no association. Furthermore, Brinkman index had a somewhat greater influence on flow-mediated dilation (R = -0.42, p = 0.008). Although subjects who smoked tended to have a larger AscAd compared with non-smokers (AscA/BSA: 17.3 ± 1.8 versus 15.2 ± 3.0 mm/m(2), p = 0.013), there was no significant change in flow-mediated dilation, suggesting that smoking might affect aortic dilation via an independent pathway. Common atherogenic risks, such as impairment of flow-mediated dilation and smoking status, affected aortic dilation in subjects with Marfan syndrome.
authors.affiliation: Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
authors.forename: Munenori
authors.initials: M
authors.lastname: Takata
authors.forename: Eisuke
authors.initials: E
authors.lastname: Amiya
authors.forename: Masafumi
authors.initials: M
authors.lastname: Watanabe
authors.forename: Kazuko
authors.initials: K
authors.lastname: Omori
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Imai
authors.forename: Daishi
authors.initials: D
authors.lastname: Fujita
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishimura
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Kato
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Morota
authors.forename: Kan
authors.initials: K
authors.lastname: Nawata
authors.forename: Atsuko
authors.initials: A
authors.lastname: Ozeki
authors.forename: Aya
authors.initials: A
authors.lastname: Watanabe
authors.forename: Shuichi
authors.initials: S
authors.lastname: Kawarasaki
authors.forename: Yumiko
authors.initials: Y
authors.lastname: Hosoya
authors.forename: Tomoko
authors.initials: T
authors.lastname: Nakao
authors.forename: Koji
authors.initials: K
authors.lastname: Maemura
authors.forename: Ryozo
authors.initials: R
authors.lastname: Nagai
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Hirata
authors.forename: Issei
authors.initials: I
authors.lastname: Komuro
mesh_terms: D000328:Adult; D001011:Aorta / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D004730:Endothelium, Vascular / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D008875:Middle Aged; D015999:Multivariate Analysis; D012039:Regional Blood Flow; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects; D013314:Stress, Mechanical; D014463:Ultrasonography; D014664:Vasodilation; D055815:Young Adult