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Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome

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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/m2, 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.

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Acknowledgments

This research was supported by the Japan Society for the Promotion of Science (JSPS) through the Funding Program for World-Leading Innovative R&D on Science and Technology (FIRST) program.

Conflict of interest

The authors declare no conflict of interests.

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Authors and Affiliations

  1. Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan

    Munenori Takata, Eisuke Amiya, Masafumi Watanabe, Kazuko Omori, Yasushi Imai, Daishi Fujita, Hiroshi Nishimura, Masayoshi Kato, Atsuko Ozeki, Aya Watanabe, Shuichi Kawarasaki, Yumiko Hosoya, Tomoko Nakao, Ryozo Nagai, Yasunobu Hirata & Issei Komuro

  2. Department of Laboratory Medicine, Teikyo University School of Medical Technology, Tokyo, Japan

    Kazuko Omori

  3. Department of Cardiothoracic Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan

    Tetsuro Morota & Kan Nawata

  4. Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan

    Koji Maemura

  5. Jichi Medical School, Tochigi, Japan

    Ryozo Nagai

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  1. Munenori Takata
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Correspondence to Masafumi Watanabe.

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M. Takata and E. Amiya contributed equally to this work.

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Takata, M., Amiya, E., Watanabe, M. et al. Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome. Heart Vessels 29, 478–485 (2014). https://doi.org/10.1007/s00380-013-0393-3

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  • DOI: https://doi.org/10.1007/s00380-013-0393-3

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