Egbe Alexander C, Connolly Heidi M, Poterucha Joseph T, Warnes Carole A
Divisions of *Cardiovascular Diseases, Mayo Clinic Rochester, Minn, USA.
Pediatric Cardiology, Mayo Clinic Rochester, Minn, USA.
Congenit Heart Dis. 2017 Jan;12(1):24-31. doi: 10.1111/chd.12391. Epub 2016 Jul 4.
There is a paucity of data about mixed aortic valve disease (MAVD) in patients with bicuspid/unicuspid aortic valve (BAV). This study sought to describe the outcomes of patients with moderate/severe MAVD.
We queried our database for patients with BAV and moderate/severe MAVD seen between 1994 and 2013. We excluded patients with baseline New York Heart Association (NYHA) III/IV symptoms, left ventricular ejection fraction <50%, aortic dimension >50 mm, and significant disease of other valves. The purpose of the study was to determine the freedom from NYHA III/IV symptoms and aortic valve replacement (AVR).
We identified 138 patients with moderate/severe MAVD; mean age was 51 ± 12 years; 112 (81%) were males; and follow-up was 8.5 ± 4 years. Ninety-two patients (67%) underwent AVR within 3.7 ± 2.5 years. Mechanical prostheses were implanted in 73 patients (79%); 22 patients (26%) and 36 patients (39%) had concomitant coronary artery bypass graft and aorta replacement during AVR respectively. There were no surgical deaths. Freedom from AVR was 84%, 51%, and 20% at 1, 5 and 10 years respectively. Predictors of AVR were age at presentation (hazard ratio [HR] 5.22; confidence interval [CI] 3.10 to 6.64) for every decade increase in age; and having severe stenosis or regurgitation at the time of presentation (HR 1.32; CI 1.05 to 3.16).
Age and disease severity should be incorporated in the risk assessment of BAV patients with MAVD, and patients with both risk factors should be monitored closely.
关于二叶式/单叶式主动脉瓣（BAV）患者的混合性主动脉瓣疾病（MAVD）的数据较少。本研究旨在描述中重度MAVD患者的结局。
我们在数据库中查询了1994年至2013年间诊断为BAV且患有中重度MAVD的患者。我们排除了基线时纽约心脏协会（NYHA）III/IV级症状、左心室射血分数<50%、主动脉内径>50 mm以及其他瓣膜有严重病变的患者。本研究的目的是确定无NYHA III/IV级症状和无需进行主动脉瓣置换（AVR）的情况。
我们确定了138例中重度MAVD患者；平均年龄为51±12岁；112例（81%）为男性；随访时间为8.5±4年。92例患者（67%）在3.7±2.5年内接受了AVR。73例患者（79%）植入了机械瓣膜；22例患者（26%）和36例患者（39%）在AVR期间分别同时进行了冠状动脉搭桥术和主动脉置换术。无手术死亡病例。1年、5年和10年时无需进行AVR的比例分别为84%、51%和20%。AVR的预测因素包括就诊时的年龄（每增加十岁，风险比[HR]为5.22；置信区间[CI]为3.10至6.64）；以及就诊时存在严重狭窄或反流（HR为1.32；CI为1.05至3.16）。
年龄和疾病严重程度应纳入BAV合并MAVD患者的风险评估中，具有这两个危险因素的患者应密切监测。
