先天二叶式主动脉瓣畸形合并二尖瓣反流病因分析

Etiology of mitral regurgitation in patients with bicuspid aortic valve

目的探讨先天二叶式主动脉瓣畸形(BAV)患者发生二尖瓣反流的病因和危险因素。方法对2011年1月至2016年6月在复旦大学附属中山医院确诊为BAV患者的超声心动图数据进行横断面分析。结果4352例BAV患者中,中至重度二尖瓣反流256例(5.9%),其中195例(76.2%)为男性、206例(80.5%)伴有中至重度主动脉瓣功能异常、15例(5.9%)合并其他先天性心脏病。256例二尖瓣反流患者中,功能性病变158例(61.7%),年龄和中至重度主动脉瓣功能异常与其发生独立相关(P<0.001);器质性病变98例(38.3%),主动脉窦部扩张与其发生独立相关(P=0.012)。二尖瓣脱垂是器质性二尖瓣反流最常见的病因(63例,64.29%),其中Barlow病占19.0%(12/63);Barlow病患者主动脉窦部内径和二尖瓣前叶脱垂比例均大于非Barlow病患者(P<0.05)。结论BAV患者发生二尖瓣反流的病因复杂,以功能性更为多见,其发生与年龄和主动脉瓣功能异常有关;器质性二尖瓣反流病因以二尖瓣脱垂为主,其中Barlow病患者存在以二尖瓣前叶脱垂和主动脉窦部扩张为特征的综合征性表现。

Objective To explore the etiology and risk factors of mitral regurgitation in patients with bicuspid aortic valve(BAV).Methods Data from 4352 patients with BAV diagnosed by echocardiography from January 2011 to June 2016 in Zhongshan Hospital,Fudan University were retrospectively analyzed.Results Among 4352 BAV patients,256(5.9%)had moderate-to-severe mitral regurgitation,including 195(76.2%)male,206(80.5%)moderate-to-severe aortic valve dysfunction,and 15(5.9%)combined with other congenital heart diseases.Among 256 mitral regurgitation patients,functional mitral regurgitation accounted for 61.7%(158/256),with age and moderate-to-severe aortic valve dysfunction as its independent risk factors(P<0.001);organic mitral regurgitation accounted for 38.3%(98/256),with aortic root dilatation as its independent risk factor(P=0.012).Mitral valve prolapse(MVP)was the most prevalent cause of organic mitral regurgitation(63,64.29%),among which 19.0%(12/63)were Barlow disease.The aortic root diameter and the proportion of anterior leaflet prolapse were greater in BAV-MVP patients with Barlow disease than those without Barlow disease(P<0.05).Conclusion The etiology of mitral regurgitation is complex among BAV patients.Functional mitral regurgitation is more prevalent and associated with age and aortic valve dysfunction.MVP was the main cause of organic mitral regurgitation,among which Barlow disease presented syndromic traits including anterior leaflet prolapse and aortic root dilatation.