Weinsaft Jonathan W, Devereux Richard B, Preiss Liliana R, Feher Attila, Roman Mary J, Basson Craig T, Geevarghese Alexi, Ravekes William, Dietz Harry C, Holmes Kathryn, Habashi Jennifer, Pyeritz Reed E, Bavaria Joseph, Milewski Karianna, LeMaire Scott A, Morris Shaine, Milewicz Dianna M, Prakash Siddharth, Maslen Cheryl, Song Howard K, Silberbach G Michael, Shohet Ralph V, McDonnell Nazli, Hendershot Tabitha, Eagle Kim A, Asch Federico M
Weill Cornell Medical College, New York, New York.
Weill Cornell Medical College, New York, New York.
J Am Coll Cardiol. 2016 Jun 14;67(23):2744-2754. doi: 10.1016/j.jacc.2016.03.570.
Aortic dissection (AoD) is a serious complication of thoracic aortic aneurysm (TAA). Relative risk for AoD in relation to TAA etiology, incidence, and pattern after prophylactic TAA surgery are poorly understood.
This study sought to determine the incidence, pattern, and relative risk for AoD among patients with genetically associated TAA.
The population included adult GenTAC participants without AoD at baseline. Standardized core laboratory tests classified TAA etiology and measured aortic size. Follow-up was performed for AoD.
Bicuspid aortic valve (BAV) (39%) and Marfan syndrome (MFS) (22%) were the leading diagnoses in the studied GenTAC participants (n = 1,991). AoD occurred in 1.6% over 3.6 ± 2.0 years; 61% of AoD occurred in patients with MFS. Cumulative AoD incidence was 6-fold higher among patients with MFS (4.5%) versus others (0.7%; p < 0.001). MFS event rates were similarly elevated versus those in patients with BAV (0.3%; p < 0.001). AoD originated in the distal arch or descending aorta in 71%; 52% of affected patients, including 68% with MFS, had previously undergone aortic grafting. In patients with proximal aortic surgery, distal aortic size (descending thoracic, abdominal aorta) was larger among patients with AoD versus those without AoD (both p < 0.05), whereas the ascending aorta size was similar. Conversely, in patients without previous surgery, aortic root size was greater in patients with subsequent AoD (p < 0.05), whereas distal aortic segments were of similar size. MFS (odds ratio: 7.42; 95% confidence interval: 3.43 to 16.82; p < 0.001) and maximal aortic size (1.86 per cm; 95% confidence interval: 1.26 to 2.67; p = 0.001) were independently associated with AoD. Only 4 of 31 (13%) patients with AoD had pre-dissection images that fulfilled size criteria for prophylactic TAA surgery at a subsequent AoD site.
Among patients with genetically associated TAA, MFS augments risk for AoD even after TAA grafting. Although increased aortic size is a risk factor for subsequent AoD, events typically occur below established thresholds for prophylactic TAA repair.
主动脉夹层（AoD）是胸主动脉瘤（TAA）的一种严重并发症。对于与TAA病因、发病率以及预防性TAA手术后的发病模式相关的AoD相对风险，人们了解甚少。
本研究旨在确定基因相关TAA患者中AoD的发病率、发病模式和相对风险。
研究人群包括基线时无AoD的成年GenTAC参与者。标准化的核心实验室检查对TAA病因进行分类并测量主动脉大小。对AoD进行随访。
在所研究的GenTAC参与者（n = 1991）中，二叶式主动脉瓣（BAV）（39%）和马凡综合征（MFS）（22%）是主要诊断。在3.6±2.0年期间，AoD发生率为1.6%；61%的AoD发生在MFS患者中。MFS患者的累积AoD发生率（4.5%）比其他患者（0.7%；p < 0.001）高6倍。与BAV患者（0.3%；p < 0.001）相比，MFS的事件发生率同样升高。71%的AoD起源于主动脉弓远端或降主动脉；52%的受影响患者，包括68%的MFS患者，此前曾接受主动脉移植。在进行近端主动脉手术的患者中，发生AoD的患者与未发生AoD的患者相比，远端主动脉大小（降胸主动脉、腹主动脉）更大（p均<0.05），而升主动脉大小相似。相反，在未进行过手术的患者中，随后发生AoD的患者主动脉根部大小更大（p < 0.05），而远端主动脉节段大小相似。MFS（比值比：7.42；95%置信区间：3.43至16.82；p < 0.001）和最大主动脉大小（每厘米1.86；95%置信区间：1.26至2.67；p = 0.001）与AoD独立相关。在31例AoD患者中，只有4例（13%）在随后的AoD部位有符合预防性TAA手术大小标准的夹层前影像。
在基因相关TAA患者中，即使在进行TAA移植后，MFS也会增加AoD风险。虽然主动脉大小增加是随后发生AoD的一个危险因素，但事件通常发生在预防性TAA修复既定阈值以下。
