Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.
Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.
复制标题
马凡综合征患者血流介导的扩张受损与主动脉扩张相关。
DOI：
10.1007/s00380-013-0393-3
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发表时间：
2014
期刊：
影响因子：
1.5
通讯作者：
Komuro I.
中科院分区：
文献类型：
--
作者：
Takata M;Amiya E;Watanabe M;Omori K;Imai Y;Fujita D;Nishimura H;Kato M;Morota T;Nawata K;Ozeki A;Watanabe A;Kawarasaki S;Hosoya Y;Nakao T;Maemura K;Nagai R;Hirata Y;Komuro I.
马凡综合征是一种以微纤维结缔组织蛋白基因异常为特征的遗传性疾病。内皮功能障碍被认为是导致二叶式主动脉瓣受试者的主动脉扩张;然而，内皮功能障碍和内皮损伤因子在马凡综合征中的作用尚未阐明。在39例马凡综合征患者中评价了血流介导的舒张功能，这是一种无创性的内皮功能测量方法。通过超声心动图测量主动脉瓣环、主动脉窦根部、窦管交界处和升主动脉处的主动脉直径，并根据体表面积（BSA）进行校正。血流介导的扩张平均值为6.5 ± 2.4%。血流介导的扩张与升胸主动脉直径（AscAd）/BSA呈负相关（R=-0.39，p = 0.020），多变量分析显示，血流介导的扩张是预测AscAd/BSA的独立因素，而主动脉的其他节段没有相关性。此外，Brinkman指数对血流介导的扩张有更大的影响（R=-0.42，p = 0.008）。尽管吸烟受试者的AscAd往往大于非吸烟受试者（AscA/BSA：17.3 ± 1.8 vs 15.2 ± 3.0 mm/m2，p= 0.013），但血流介导的扩张无显著变化，表明吸烟可能通过独立途径影响主动脉扩张。常见的致动脉粥样硬化风险，如血流介导的扩张受损和吸烟状况，影响马凡综合征受试者的主动脉扩张。
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.