Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands2Department of Cardiology, National University Heart Center, National University Health System, Singapore.
Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
JAMA Cardiol. 2017 Mar 1;2(3):285-292. doi: 10.1001/jamacardio.2016.5228.
Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality.
To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality).
DESIGN, SETTING, AND PARTICIPANTS: In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study.
Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction.
Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection.
Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447 [24.4%] at 5 years) vs patients without raphe (30 [14.0%] at 1 year, 32 [15.0%] at 2 years, and 40 [18.0%] at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 [5.1%] at 1 year, 87 [6.2%] at 2 years, and 110 [9.5%] at 5 years) vs patients without raphe (2 [1.8%] at 1 year, 3 [3.0%] at 2 years, and 5 [4.4%] at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality.
In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.
重要性：关于二叶式主动脉瓣（BAV）形态学发现与瓣叶功能障碍的严重程度、主动脉病变的存在以及包括主动脉瓣手术、主动脉夹层和全因死亡率在内的并发症之间的关系，人们知之甚少。
目的：研究 BAV 形态学发现（嵴对非嵴）与瓣叶功能障碍、主动脉病变和预后（包括主动脉瓣手术、主动脉夹层和全因死亡率的需要）之间的关系。
设计、地点和参与者：在这项对在三级转诊中心接受治疗的 BAV 患者的大型国际多中心注册研究中，评估了 2118 例 BAV 患者。研究纳入了 1991 年 6 月 1 日至 2015 年 11 月 31 日期间因超声心动图检查而转诊的患者。
暴露因素：回顾性分析临床和超声心动图数据。根据 Sievers 和 Schmidtke 分类，将 BAV 的形态学发现分类。主动脉瓣功能分为正常、反流或狭窄。BAV 主动脉病变的模式包括以下几种：1 型，升主动脉和主动脉根部扩张；2 型，仅升主动脉扩张；3 型，仅窦部扩张和/或窦管交界处扩张。
主要结果和措施：分析嵴的存在和位置与严重（中度和重度）主动脉瓣功能障碍和主动脉扩张和/或夹层的风险之间的关系。
结果：在 2118 例患者（平均[标准差]年龄 47[18]岁；1525[72.0%]为男性）中，1881 例（88.8%）为融合嵴的二叶式主动脉瓣，237 例（11.2%）为无嵴的二叶式主动脉瓣。有嵴的二叶式主动脉瓣瓣叶功能障碍的患病率明显更高，主动脉瓣反流的发生率明显更高（622[33.1%]比 57[24.1%]，P<.001），主动脉瓣狭窄的发生率也明显更高（728[38.7%]比 51[21.5%]，P<.001）。此外，有嵴的二叶式主动脉瓣患者主动脉瓣置换事件发生率明显更高（1 年时 364[19.9%]，2 年时 393[21.4%]，5 年时 447[24.4%]），而无嵴的二叶式主动脉瓣患者发生率分别为 30[14.0%]、32[15.0%]和 40[18.0%]（P=.02）。此外，有嵴的二叶式主动脉瓣患者全因死亡率明显更高（1 年时 77[5.1%]，2 年时 87[6.2%]，5 年时 110[9.5%]），而无嵴的二叶式主动脉瓣患者发生率分别为 2[1.8%]、3[3.0%]和 5[4.4%]（P=.03）。然而，多变量分析显示，嵴的存在与全因死亡率无显著相关性。
结论和相关性：在这项大型多中心国际 BAV 注册研究中，嵴的存在与严重主动脉瓣狭窄和反流的发生率更高相关。嵴的存在也与主动脉瓣和主动脉手术的发生率增加相关。尽管有嵴的二叶式主动脉瓣患者的死亡率高于无嵴的患者，但嵴的存在与全因死亡率的增加并无独立相关性。
