Lau Christopher, Soletti Giovanni, Weinsaft Jonathan W, Rahouma Mohamed, Al Zghari Talal, Olaria Roberto Perezgrovas, Harik Lamia, Yaghmour Mohammad, Dimagli Arnaldo, Gaudino Mario, Girardi Leonard N
Department of Cardiothoracic Surgery, Weill Cornell Medicine, New York, NY.
Department of Cardiothoracic Surgery, Weill Cornell Medicine, New York, NY.
J Thorac Cardiovasc Surg. 2023 Dec;166(6):1548-1557.e2. doi: 10.1016/j.jtcvs.2023.03.029. Epub 2023 May 8.
To compare operative and long-term outcomes between patients with and without Marfan syndrome undergoing thoracoabdominal aortic aneurysm repair.
We identified all consecutive patients undergoing thoracoabdominal aortic aneurysm repair between 1997 and 2022. Primary outcome was composite of major adverse events. Secondary outcomes were individual complications and long-term survival. Inverse probability of treatment weighting was performed. Weighted Kaplan-Meier curves were used to estimate long-term survival. Multivariable analysis identified factors associated with major adverse events.
Six hundred eighty-four patients underwent open thoracoabdominal aortic aneurysm repair. Ninety (13.1%) had Marfan syndrome, whereas 594 (86.9%) did not. Marfan patients were younger (46 years [range, 36-56 years] vs 69 years [range, 61-76 years]; P < .001). Extent II or III aneurysms (57 out of 90 [63.3%] vs 211 out of 594 [35.6%]; P < .001) and type I or III chronic dissection (77 out of 90 [85.3%] vs 242 out of 594 [40.8%]; P < .001) were more common. Cardiovascular risk factors were less frequent in Marfan patients. There was no difference in major adverse events between groups (12 out of 90 [13.3%] vs 100 out of 594 [16.8%]; P = .49). Operative mortality was similar between groups (3 out of 90 [3.3%] vs 28 out of 594 [4.7%]; P = .75). Unweighted survival at 10 years was 78.7% vs 46.8% (P = .001). Weighted Kaplan-Meier curves showed no difference in long-term survival (adjusted hazard ratio, 0.79; 95% CI, 0.32-1.99; P = .62; Log-rank P = .12). At multivariable analysis, renal insufficiency (odds ratio, 2.29; 95% CI, 1.43-3.68; P < .01) and urgent/emergency procedure (odds ratio, 2.17; 95% CI, 1.35-3.48; P < .01) were associated with major adverse events, whereas Marfan syndrome was not (odds ratio, 1.56; 95% CI, 0.69-3.49; P = .28).
Open thoracoabdominal aortic aneurysm repair can be performed with similar operative outcomes in patients with and without Marfan syndrome despite differing risk profiles. Operative/perioperative strategies must be tailored to specific needs of each patient to optimize outcomes.
比较患有和未患马凡综合征的患者在接受胸腹主动脉瘤修复术后的手术效果和长期预后。
我们确定了1997年至2022年间所有连续接受胸腹主动脉瘤修复术的患者。主要结局是主要不良事件的综合情况。次要结局是个体并发症和长期生存率。进行了治疗权重的逆概率分析。采用加权Kaplan-Meier曲线来估计长期生存率。多变量分析确定了与主要不良事件相关的因素。
684例患者接受了开放性胸腹主动脉瘤修复术。90例（13.1%）患有马凡综合征，而594例（86.9%）未患。马凡综合征患者更年轻（46岁[范围36 - 56岁] 对比69岁[范围61 - 76岁]；P <.001）。Ⅱ型或Ⅲ型动脉瘤（90例中的57例[63.3%] 对比594例中的211例[35.6%]；P <.001）以及Ⅰ型或Ⅲ型慢性夹层（90例中的77例[85.3%] 对比594例中的242例[40.8%]；P <.001）更为常见。马凡综合征患者的心血管危险因素较少见。两组之间主要不良事件无差异（90例中的12例[13.3%] 对比594例中的100例[16.8%]；P =.49）。两组的手术死亡率相似（90例中的3例[3.3%] 对比594例中的28例[4.7%]；P =.75）。未加权的10年生存率分别为78.7%和46.8%（P =.001）。加权Kaplan-Meier曲线显示长期生存率无差异（调整后的风险比为0.79；95%置信区间为0.32 - 1.99；P =.62；对数秩检验P =.12）。在多变量分析中，肾功能不全（比值比为2.29；95%置信区间为1.43 - 3.68；P <.01）和急诊/紧急手术（比值比为2.17；95%置信区间为1.35 - 3.48；P <.01）与主要不良事件相关，而马凡综合征则无关（比值比为1.56；95%置信区间为0.69 - 3.49；P =.28）。
尽管风险特征不同，但患有和未患马凡综合征的患者在接受开放性胸腹主动脉瘤修复术时，手术效果相似。手术/围手术期策略必须根据每个患者的具体需求进行调整，以优化预后。
