二叶式主动脉瓣基础与临床.PDF

临床与病理杂志 2017, 37(10) J Clin Path ol R e s 2243 doi: 10.3978/j.issn.2095-6959.2017.10.036 View this article at: /10.3978/j.issn.2095-6959.2017.10.036 二叶式主动脉瓣基础与临床 蔡怀敏 综述 王春生，孙晓宁 审校 (复旦大学附属中山医院心脏外科，上海 200032) [摘　要] 二叶式主动脉瓣 ( b i c u s p i d a o r t i c v a l v e ，B AV ) 是目前最常见的先天性心脏病，患病率预计为 0.5%~2% ，男性患者与女性患者的比例约为3 : 1 。可以单独发生，也可以伴随其他的先天性心血管 病。BAV 患者常出现主动脉瓣返流、主动脉瓣狭窄、主动脉扩张、主动脉瘤、主动脉夹层、血栓 和感染性心内膜炎等并发症。BAV 的瓣膜可有正常的功能，也可产生钙化、狭窄或者关闭不全等 功能紊乱。 [关键词] 二叶式主动脉瓣；基因；并发症；主动脉扩张 Fundamental and clinical study of bicuspid aortic valve CAI Huaimin, WANG Chunsheng, SUN Xiaoning (Department of Cardiac Surgery, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200032, China) Abstract Bicuspid aortic valve (BAV) disease is the most common congenital cardiac defect, occurring in 0.5%–2% of the general population. It is almost 3 times more common in males than females. BAV can occur alone. Moreover, BAV may be associated with other cardiovascular anomalies. Complications associated with BAV include aortic stenosis (AS) and regurgitation, aortic dilation and dissection, thrombus and infective endocarditis. This review summarizes current understanding of anatomy, genetics, and clinical aspects of BAV disease. Keywords bicuspid aortic valve; gene; complication; aortic dilation 二叶式主动脉瓣 (bicuspid aortic valve ，BAV)通 展。尽管症状通常出现在成年后，BAV却可以从有 常是在健康或无症状的人群中发现，但它却伴随着 严重宫内疾病胎儿到无症状老年人中发现。 严重的、长期的风险，包括进展性主动脉瓣疾病 (狭窄或返流 ) 、胸主动脉瘤和主动脉夹层等。BAV 1 形态 有较高的遗传倾向性。BAV 的遗传倾向性和人群筛 查推荐都应被重点强调。基因型、环境危险因素和 正常的