Department of Cardiac Surgery, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Department of Cardiovascular Surgery, University Heart Centre Freiburg - Bad Krozingen, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Eur J Cardiothorac Surg. 2024 Mar 1;65(3). doi: 10.1093/ejcts/ezae069.
The aim of this study was to report on mid-term outcomes after endovascular aortic repair (EVAR) in patients with Marfan (MFS) or Loeys-Dietz (LDS) syndrome.
We analysed data from 2 European centres of patients with MFS and LDS undergoing EVAR. Patients were analysed based on (i) timing of the procedure (planned versus emergency procedure) and (ii) the nature of the landing zone (safe versus non-safe). The primary end-point was freedom from reintervention. Secondary end-points were freedom from stroke, bleeding and death.
A population of 419 patients with MFS (n = 352) or LDS (n = 67) was analysed for the purpose of this study. Thirty-nine patients (9%) underwent EVAR. Indications for thoracic endovascular aortic repair or EVAR were aortic dissection in 13 (33%) patients, aortic aneurysm in 22 (57%) patients and others (intercostal patch aneurysm, penetrating atherosclerotic ulcer, pseudoaneurysm, kinking of frozen elephant trunk (FET)) in 4 (10%) patients. Thoracic endovascular repair was performed in 34 patients, and abdominal endovascular aortic repair was performed in 5 patients. Mean age at 1st thoracic endovascular aortic repair/EVAR was 48.5 ± 15.4 years. Mean follow-up after 1st thoracic endovascular aortic repair/EVAR was 5.9 ± 4.4 years. There was no statistically significant difference in the rate of reinterventions between patients with non-safe landing zone and the patients with safe proximal landing zone (P = 0.609). Furthermore, there was no increased probability for reintervention after planned endovascular intervention compared to emergency procedures (P = 0.916). Mean time to reintervention, either open surgical or endovascular, after planned endovascular intervention was in median 3.9 years (95% confidence interval 2.0-5.9 years) and 2.0 years (95% confidence interval -1.1 to 5.1 years) (P = 0.23) after emergency procedures.
EVAR in patients with MFS and LDS and a safe landing zone is feasible and safe. Endovascular treatment is a viable option when employed by a multi-disciplinary aortic team even if the landing zone is in native tissue.
本研究旨在报告马凡氏综合征（MFS）或洛伊兹-迪茨综合征（LDS）患者行血管内主动脉修复（EVAR）的中期结果。
我们分析了在 2 个欧洲中心接受 EVAR 的 MFS 和 LDS 患者的数据。根据（i）手术时机（计划与紧急手术）和（ii）着陆区性质（安全与非安全）对患者进行分析。主要终点是免于再次干预。次要终点是免于卒中、出血和死亡。
为进行本研究，分析了 419 例 MFS（n=352）或 LDS（n=67）患者的资料。39 例（9%）患者行 EVAR。13 例（33%）患者行胸主动脉腔内修复术或 EVAR 的指征为主动脉夹层，22 例（57%）患者为主动脉瘤，4 例（10%）患者为其他病变（肋间动脉修补、穿透性粥样硬化性溃疡、假性动脉瘤、冷冻象鼻吻合口扭结（FET））。34 例患者行胸主动脉腔内修复术，5 例患者行腹主动脉腔内修复术。首次胸主动脉腔内修复术/EVAR 时的平均年龄为 48.5±15.4 岁。首次胸主动脉腔内修复术/EVAR 后平均随访时间为 5.9±4.4 年。非安全近端着陆区与安全近端着陆区患者的再干预率无统计学差异（P=0.609）。此外，与急诊手术相比，计划性血管内介入后行血管内介入的再干预概率无增加（P=0.916）。计划性血管内介入后，开放手术或血管内再次干预的中位时间分别为 3.9 年（95%置信区间 2.0-5.9 年）和 2.0 年（95%置信区间 -1.1-5.1 年）（P=0.23）。
MFS 和 LDS 患者安全着陆区行 EVAR 是可行且安全的。即使着陆区位于原生组织，多学科主动脉团队采用血管内治疗也是可行的选择。
