先天性二叶式主动脉瓣畸形与主动脉病变的研究进展  

Research Progress of Bicuspid Aortic Valve Aortopathy

作　　者：刘鼎乾 孙逸平 李军 赖颢 王春生 LIU Ding-qian;SUN Yi-ping;LI Jun;LAI Hao;WANG Chun-sheng(Department of Cardiac Surgery,Shanghai Institute of Cardiovascular Diseases,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院心外科上海市心血管病研究所,上海市200032

出　　处：《中国分子心脏病学杂志》2019年第6期3169-3171,共3页Molecular Cardiology of China

基　　金：国家自然科学基金(81570422,81500194)。

摘　　要：先天性二叶式主动脉瓣畸形(BAV)是成人常见的先天性心脏畸形,无论是否合并瓣膜功能异常,BAV被认为与主动脉病变相关。目前研究表明,分子生物学及血流动力学是BAV引起以主动脉扩张为主病变的主要因素。在瓣膜发育过程中相关通路的表达异常导致BAV形成。不同瓣膜融合类型的BAV导致的异常血流模式有所不同。本文就近年来针对先天性二叶式主动脉瓣畸形引起主动脉病变相关的机制进行综述,探讨其对于BAV主动脉病变相关临床诊疗的意义。Bicuspid aortic valve(BAV)is a common congenital heart defect in adults.It has been proven that BAV is related with aortopathy regardless of valve complications.By far,genetic and hemodynamic bases are presumed to be major contributors to the bicuspid aortopathy which leads to aortic dilation.Abnormalities of valve formation molecular pathway leads to BAV.The pattern of abnormal hemodynamic flow and aortic dilatation differs with valve fusion type.This article reviewed advances of bicuspid aortopathy researches and progress of clinical treatments.

关 键 词：先天性二叶式主动脉瓣畸形 主动脉病变 

分 类 号：R542.52[医药卫生—心血管疾病] R543.1[医药卫生—内科学]