Song Shinjeong, Seo Jiwon, Cho Iksung, Hong Geu-Ru, Ha Jong-Won, Shim Chi Young
Division of Cardiology, Department of Internal Medicine, Ewha Womans University Hospital, Ewha Womans University College of Medicine, Seoul, South Korea.
Division of Cardiology, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, South Korea.
Front Cardiovasc Med. 2021 Jan 11;7:603323. doi: 10.3389/fcvm.2020.603323. eCollection 2020.
Using echocardiographic surveillance, many patients are diagnosed with bicuspid aortic valve (BAV) without significant valve dysfunction. Limited data are available regarding the progression and outcomes of non-dysfunctional BAV. We investigated 1,307 BAV patients (984 male, mean age 56 years) diagnosed from Jan 2003 through Dec 2018 in a single tertiary center. Seven hundred sixty-one patients underwent follow-up echocardiography at ≥1 year post-diagnosis. Non-dysfunctional BAV was defined as BAV without moderate aortic stenosis (AS) or aortic regurgitation (AR). The presence of aortopathy was defined as an ascending aorta diameter >37mm. Progression to significant BAV dysfunction, progression to severe aortopathy (ascending aorta diameter ≥45mm), and incidence of valve or aorta operation were analyzed. One hundred eighty-seven (25%) patients showed non-dysfunctional BAV. Among them, 104 (56%) had mild AS or AR, and 81 (43%) had aortopathy at indexed echocardiography. At 6.0 ± 3.8 years post-diagnosis, 56 (29%) progressed to dysfunctional BAV, 28 (15%) progressed to severe aortopathy, 22 (12%) underwent valve operation, and 19 (10%) experienced aorta operation. Eighty-nine percent of patients with normal BAV function and 61% of patients with mild AS or AR maintained non-dysfunctional BAV. More patients with aortopathy progressed to severe aortopathy (35 vs. 0% without aortopathy, < 0.001), with a higher incidence of aorta operation (21 vs. 2%, < 0.001). In patients with non-dysfunctional BAV, initial BAV function and degree of aorta dilatation might be important for progression and outcomes. Patients without any dysfunction or aortopathy tend to maintain good structure and function for 6 years.
通过超声心动图监测，许多患者被诊断为二叶式主动脉瓣（BAV），但瓣膜功能无明显障碍。关于无功能障碍的BAV的进展和结局的数据有限。我们调查了2003年1月至2018年12月在单个三级中心诊断出的1307例BAV患者（984例男性，平均年龄56岁）。761例患者在诊断后≥1年接受了随访超声心动图检查。无功能障碍的BAV定义为无中度主动脉瓣狭窄（AS）或主动脉瓣反流（AR）的BAV。主动脉病变的存在定义为升主动脉直径> 37mm。分析了进展为严重BAV功能障碍、进展为严重主动脉病变（升主动脉直径≥45mm）以及瓣膜或主动脉手术的发生率。187例（25％）患者表现为无功能障碍的BAV。其中，104例（56％）有轻度AS或AR，81例（43％）在超声心动图检查时有主动脉病变。在诊断后6.0±3.8年，56例（29％）进展为功能障碍性BAV，28例（15％）进展为严重主动脉病变，22例（12％）接受了瓣膜手术，19例（10％）接受了主动脉手术。BAV功能正常的患者中有89％以及轻度AS或AR的患者中有61％维持无功能障碍的BAV。更多有主动脉病变的患者进展为严重主动脉病变（35％对无主动脉病变者的0％，<0.001），主动脉手术的发生率更高（21％对2％，<0.001）。在无功能障碍的BAV患者中，初始BAV功能和主动脉扩张程度可能对进展和结局很重要。没有任何功能障碍或主动脉病变的患者倾向于在6年内保持良好的结构和功能。
