Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy
Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy
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DOI：
10.1001/jamacardio.2016.5228
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发表时间：
2017-03-01
期刊：
影响因子：
24
通讯作者：
Bax, Jeroen J.
中科院分区：
文献类型：
--
作者：
Kong, William K. F.;Delgado, Victoria;Bax, Jeroen J.
二尖瓣病变(BAV)的形态表现与瓣膜功能不全的程度、主动脉病变的存在以及并发症(包括主动脉瓣手术、主动脉夹层和全因死亡率)之间的关系知之甚少。目的探讨BAV形态表现(中缝与非中缝)与瓣膜功能障碍的程度、存在的主动脉病变和预后(包括是否需要进行主动脉瓣手术、主动脉夹层和全因死亡率)之间的关系。研究对象为1991年6月1日至2015年11月31日接受超声心动图检查的患者，对其临床和超声心动图资料进行回顾性分析。根据Siever和Schmidtke的分类对BAV的形态表现进行分类。主动脉瓣功能分为正常、反流或狭窄。BAV病变的类型包括：1型，升主动脉和主动脉根部扩张；2型，升主动脉孤立性扩张；3型，Valsalva窦和/或窦管交界处孤立性扩张。中缝的存在和位置与严重(中、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主要结果和措施。结果2118名患者(平均[SD]年龄，47[18]岁；合并中缝1525例(72.0%)，合并中缝的1881例(88.8%)，未合并中缝的237例(11.2%)。存在RAPHE的二叶型主动脉瓣瓣膜功能不全的发生率显著更高，主动脉瓣关闭不全的发生率(622[33.1%]比57[24.1%]，P<；.001)和主动脉狭窄的发生率(728[38.7%]比51[21.5%]，P<；.001)显著增加。此外，有RAPHE的BAV患者的主动脉瓣置换事件发生率(1年364例[19.9%]，2年393例[21.4%]，5年447例[24.4%])显著高于无RAPHE的患者(1年30例[14.0%]，2年32例[15.0%]，5年40例[18.0%])(P=0.02)。此外，有RAPHE的BAV患者的全因死亡事件发生率(1年77例[5.1%]，2年87例[6.2%]，5年110例[9.5%])显著高于无RAPHE的患者(1年2例[1.8%]，2年3例[3.0%]，5年5例[4.4%])(P=0.03)。然而，在多变量分析中，RAPHE的存在与全因死亡率并不显著相关。结论和相关性：在这个大型的多中心国际BAV登记系统中，RAPHE的存在与显著的主动脉狭窄和反流的发生率较高相关。RAPHE的存在也与主动脉瓣和主动脉手术的发生率增加有关。尽管有BAV和RAPH的患者比没有BAV和RAPH的患者有更高的死亡率，但RAPH的存在并不独立地与全因死亡率的增加有关。版权所有2017美国医学会。版权所有。
IMPORTANCE 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.OBJECTIVE 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.EXPOSURES 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.MAIN OUTCOMES AND MEASURES 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.RESULTS 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.CONCLUSIONS AND RELEVANCE 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. Copyright 2017 American Medical Association. All rights reserved.