Straneo Pablo, Parma Gabriel, Lluberas Natalia, Marichal Alvaro, Soca Gerardo, Cura Leandro, Paganini Juan J, Brusich Daniel, Florio Lucia, Dayan Victor
Department of Cardiology, University Hospital, Montevideo, Uruguay.
Asian Cardiovasc Thorac Ann. 2017 Mar;25(3):192-198. doi: 10.1177/0218492317696375. Epub 2017 Jan 1.
Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m, p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = -0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.
二叶式主动脉瓣患者发生主动脉扩张的风险增加。一氧化氮合酶缺乏被认为是致病因素。在二叶式主动脉瓣且主动脉直径正常的患者中，尚未进行血流介导的血管舒张与主动脉直径之间的相关性研究。作为一种遗传性疾病，我们比较了这些患者及其一级亲属的超声心动图特征和内皮功能。方法：对18例二叶式主动脉瓣患者（14例1型和4例2型）及其19名一级亲属进行了全面体格检查、常规实验室检查、经胸超声心动图检查以及内皮依赖性和非依赖性血流介导的血管舒张测量。结果：一级亲属较年轻（36.7±18.8岁对50.5±13.9岁，p = 0.019），射血分数较高（64.6%±1.7%对58.4%±9.5%，p = 0.015）。两组的体表面积指数化主动脉直径相似，但二叶式主动脉瓣患者的主动脉管腔较大（19.3±2.7对vs.17.4±2.2mm·m，p = 0.033）。两组的血流依赖性血管舒张相似。在二叶式主动脉瓣患者中，非血流依赖性血管舒张与主动脉根部直径之间存在显著负相关（R = -0.57，p = 0.05）。结论：无主动脉病变的二叶式主动脉瓣患者的升主动脉直径大于其一级亲属。两组的内皮功能相似，且与升主动脉直径无关。尽管如此，二叶式主动脉瓣患者的非内皮依赖性舒张与主动脉根部直径之间存在负相关。
