Amako Mau, Spear Rafaëlle, Clough Rachel E, Hertault Adrien, Azzaoui Richard, Martin-Gonzalez Teresa, Sobocinski Jonathan, Haulon Stéphan
Aortic Center, Hôpital Cardiologique, CHRU Lille, Lille, France; Department of Cardiovascular Surgery, Fukuoka University, Fukuoka, Japan.
Aortic Center, Hôpital Cardiologique, CHRU Lille, Lille, France.
Ann Vasc Surg. 2017 Feb;39:289.e9-289.e12. doi: 10.1016/j.avsg.2016.07.069. Epub 2016 Nov 24.
The aim of this study is to describe a total endovascular aortic repair with branched and fenestrated endografts in a young patient with Marfan syndrome and a chronic aortic dissection. Open surgery is the gold standard to treat aortic dissections in patients with aortic disease and Marfan syndrome.
In 2000, a 38-year-old man with Marfan syndrome underwent open ascending aorta repair for an acute type A aortic dissection. One year later, a redo sternotomy was performed for aortic valve replacement. In 2013, the patient presented with endocarditis and pulmonary infection, which necessitated tracheostomy and temporary dialysis. In 2014, the first stage of the endovascular repair was performed using an inner branched endograft to exclude a 77-mm distal arch and descending thoracic aortic aneurysm. In 2015, a 63-mm thoracoabdominal aortic aneurysm was excluded by implantation of a 4-fenestrated endograft. Follow-up after both endovascular repairs was uneventful.
Total aortic endovascular repair was successfully performed to treat a patient with arch and thoraco-abdominal aortic aneurysm associated with chronic aortic dissection and Marfan syndrome. The postoperative images confirmed patency of the endograft and its branches, and complete exclusion of the aortic false lumen.
Endovascular repair is a treatment option in patients with connective tissue disease who are not candidates for open surgery. Long-term follow-up is required to confirm these favorable early outcomes.
本研究旨在描述在一名患有马凡综合征和慢性主动脉夹层的年轻患者中使用分支型和开窗型腔内移植物进行全腔内主动脉修复。开放手术是治疗患有主动脉疾病和马凡综合征患者主动脉夹层的金标准。
2000年，一名38岁的马凡综合征男性因急性A型主动脉夹层接受了升主动脉开放修复术。一年后，因主动脉瓣置换术进行了再次胸骨切开术。2013年，该患者出现心内膜炎和肺部感染，需要进行气管切开术和临时透析。2014年，使用内置分支型腔内移植物进行了第一阶段的腔内修复，以排除一个77毫米的远端主动脉弓和降主动脉瘤。2015年，通过植入一个四开窗型腔内移植物排除了一个63毫米的胸腹主动脉瘤。两次腔内修复后的随访均无异常。
成功进行了全主动脉腔内修复，以治疗一名患有与慢性主动脉夹层和马凡综合征相关的主动脉弓和胸腹主动脉瘤的患者。术后影像证实了腔内移植物及其分支的通畅，以及主动脉假腔的完全封闭。
腔内修复是不适合开放手术的结缔组织病患者的一种治疗选择。需要长期随访以证实这些良好的早期结果。
