先天二叶式主动脉瓣畸形临床及超声心动图特征分析

Analysis of congenital bicuspid aortic valve malformation clinical and echocardiographic features

目的:探讨先天二叶式主动脉瓣畸形(BAV)患者主动脉瓣功能异常和大血管病变的分布特征和危险因素。方法:对复旦大学附属中山医院2012年1月至2015年12月确诊为BAV患者的超声心动图数据进行横断面分析。结果:共纳入BAV患者2781例,占超声心动图检查总人数的1.1%,其中70.2%为男性,60.4%伴中度以上主动脉瓣功能异常,54.2%合并升主动脉和19.7%患者主动脉窦部扩张。单纯主动脉瓣狭窄和混合性双病变的检出率随年龄增长递增(P<0.0001);单纯主动脉瓣反流的检出率随年龄增长递减(P<0.0001)。男性、左右冠瓣融合型、单纯主动脉瓣反流为患者发生主动脉窦部扩张的独立危险因素;年龄、单纯主动脉瓣狭窄和主动脉瓣混合性双病变为患者发生升主动脉扩张的独立危险因素。结论:BAV发病率高且临床表现多样,以主动脉瓣功能异常和升主动脉扩张最常见,其表型差异对治疗有指导意义。

Objective:To explore the distribution patterns and risk factors of abnomal aortic valve function and vascular complications in patients with congenital bicuspid aortic valve(BAV)malformation.Methods:Data from patients with BAV confirmed by echocardiography from January 2012 to December 2015 in our institute were retrospectively collected.Results:A total of 2781 patients with BAV were included,accounting for 1.1%of the total number of patients examined by echocardiography.Among them,70.2%were male,and the patients with moderate or higher aortic valve dysfunction accounted for 60.4%,and the patients with concomitant ascending aorta and aortic sinus dilatation accounted for 54.2%and 19.7%,respectively.The detection rate of simple aortic stenosis and mixed aortic valve dysfunction increased with age(P<0.0001),while detection rate of simple aortic regurgitation decreased with age(P<0.0001).Male gender,right-left cusp fusion type,and simple aortic regurgitation were independent risk factors for aortic root dilatation.Elder age,simple aortic stenosis,and mixed aortic valve dysfunction were independndent risk factors for ascending aortic dilatation.Conclusions:The prevalence of BAV is high and its clinical manifestations are diverse.Aortic valve dysfunction and ascending aorta dilatation are the most common,and their phenotypic differences have guiding significance for the treatment.