German Aortic Center Hamburg, Department of Vascular Medicine, University Heart & Vascular Center Hamburg, Hamburg, Germany.
J Endovasc Ther. 2022 Oct;29(5):717-723. doi: 10.1177/15266028211061276. Epub 2021 Dec 12.
The aim of this study is to report the safety and effectiveness of thoracic endovascular aortic repair (TEVAR) in a native proximal landing zone (PLZ) 2 for chronic type B aortic dissection (TBAD) patients with genetic aortic syndrome (GAS).
A retrospective review of a single center database to identify patients with GAS treated with TEVAR in native PLZ 2 for chronic TBAD and thoracic false lumen aneurysm between February 2012 and February 2018 was undertaken.
In total, 31 patients with GAS (24 Marfan syndrome [MFS], 5 Loeys-Dietz syndrome [LDS], and 2 vascular Ehlers-Danlos syndrome [vEDS]) were treated by endovascular repair. Nineteen patients were treated by TEVAR as index procedures with 8 patients (5 females, mean age = 55, range = 36-79 years old) receiving TEVAR in native PLZ 2. Left subclavian artery (LSA) perfusion was preserved in all 8 patients: by left common carotid artery-LSA bypass in 6 patients, chimney stenting of the LSA in 1 patient, and partial coverage of LSA ostium in 1 patient. Technical success was achieved in all patients (100%). There was no 30 day mortality (0%). The 30 day morbidity (0%) was free from major complications. The median follow-up was 40 months (range = 7-79). One patient died due to non-aortic-related cause. Native PLZ 2 was free from complications in MFS patients (5/8). Two patients with LDS developed type Ia endoleak with aneurysmal progression. One patient was treated by proximal extension with a double inner branched arch stent-graft landing in the replaced ascending aorta. The other one was treated with frozen elephant trunk.
Thoracic endovascular aortic repair in native PLZ 2 was safe and effective with no early or midterm PLZ complications in patients with MFS with chronic TBAD in this limited series. Native PLZ 2 is not safe in patients with LDS and should only be used in emergencies as a bridging to open repair.
本研究旨在报告在伴有遗传性主动脉综合征（GAS）的慢性 B 型主动脉夹层（TBAD）患者的原生近端锚定区（PLZ）2 中进行胸主动脉腔内修复术（TEVAR）的安全性和有效性。
对 2012 年 2 月至 2018 年 2 月期间在单一中心数据库中接受 TEVAR 治疗的伴有 GAS 的慢性 TBAD 和胸主动脉假性腔动脉瘤患者进行回顾性分析。
共 31 例 GAS 患者（24 例马凡综合征[MFS]，5 例 Loeys-Dietz 综合征[LDS]，2 例血管性埃勒斯-当洛斯综合征[vEDS]）接受了血管内修复治疗。19 例患者接受 TEVAR 作为初始治疗，其中 8 例（5 例女性，平均年龄 55 岁，范围 36-79 岁）在原生 PLZ 2 接受 TEVAR。8 例患者均保留了左锁骨下动脉（LSA）灌注：6 例患者行左颈总动脉-LSA 旁路术，1 例患者行 LSA 烟囱支架术，1 例患者行 LSA 开口部分覆盖术。所有患者均获得技术成功（100%）。无 30 天死亡（0%）。30 天发病率（0%）无重大并发症。中位随访时间为 40 个月（范围 7-79 个月）。1 例患者死于非主动脉相关原因。MFS 患者的原生 PLZ 2 无并发症（8/8）。2 例 LDS 患者发生 I 型内漏伴动脉瘤进展。1 例患者行近端延伸治疗，双内分支弓支架移植物在人工血管升主动脉中着陆。另 1 例患者行冷冻象鼻手术。
在伴有慢性 TBAD 的 MFS 患者中，在原生 PLZ 2 行 TEVAR 安全有效，在本有限系列中无早期或中期 PLZ 并发症。LDS 患者的原生 PLZ 2 不安全，只能作为紧急情况使用，作为开放修复的过渡。
