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一名患有洛伊斯-迪茨综合征的22岁个体在多次血管内治疗失败后成功进行胸腹主动脉瘤开放修复术

Successful Open Repair of a Thoracoabdominal Aortic Aneurysm After Multiple Failed Endovascular Treatments in a 22-Years-Old Individual With Loeys-Dietz Syndrome.

作者信息

Santoro Annarita, Rizk Mohamed, Inga Tavara Laura, Ramadan Moh'd Shafiq, Melissano Germano

机构信息

Department of Vascular Surgery, San Raffaele Scientific Institute, Vita-Salute University School of Medicine, Milan, Italy.

出版信息

Vasc Endovascular Surg. 2025 Feb;59(2):218-223. doi: 10.1177/15385744241285112. Epub 2024 Sep 25.

DOI:10.1177/15385744241285112
PMID:39323080
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11636019/
Abstract

Loeys-Dietz syndrome is a rare genetically triggered disease characterized by aortic involvement, predisposing individuals to aneurysm and dissection at young age. Open repair is considered the treatment of choice despite the fact that it is associated with significant morbidity and mortality rates. On the other hand, endovascular treatment may be also considered an acceptable option in specific cases such as emergency or in patients unfit for open surgery or when landing zones are within surgical grafts. We report the case of a thoracoabdominal aortic aneurysm (TAAA) open surgical repair (OSR) in a 22-year-old male patient diagnosed with type 2 Loeys-Dietz syndrome, treated by means of a TAAA replacement with a 30-mm multi-branched "Coselli" aortic graft (Vascutek, Renfrewshire, Scotland, UK) after multiple previously interventions, including a thoracic endovascular aortic repair (TEVAR) and a custom made endograft for the visceral aorta.

摘要

洛伊迪茨综合征是一种罕见的由基因引发的疾病,其特征为主动脉受累,使个体在年轻时就易患动脉瘤和主动脉夹层。尽管开放修复与显著的发病率和死亡率相关,但仍被视为首选治疗方法。另一方面,在特定情况下,如急诊、不适于开放手术的患者或着陆区位于手术移植物内时,血管内治疗也可被视为一种可接受的选择。我们报告了一例22岁男性患者的胸腹主动脉瘤(TAAA)开放手术修复(OSR)病例,该患者被诊断为2型洛伊迪茨综合征,在先前进行了包括胸主动脉腔内修复术(TEVAR)和定制内脏主动脉内支架移植物等多次干预后,采用30毫米多分支“科塞利”主动脉移植物(Vascutek,英国苏格兰伦弗鲁郡)进行TAAA置换治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/8d2e5b62459a/10.1177_15385744241285112-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/b6a9376a01ff/10.1177_15385744241285112-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/efdd1c863356/10.1177_15385744241285112-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/91e37a854c1f/10.1177_15385744241285112-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/8d2e5b62459a/10.1177_15385744241285112-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/b6a9376a01ff/10.1177_15385744241285112-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/efdd1c863356/10.1177_15385744241285112-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/91e37a854c1f/10.1177_15385744241285112-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b2e9/11636019/8d2e5b62459a/10.1177_15385744241285112-fig4.jpg

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Connective tissue disease type mediates branch patency of grafts in open thoracoabdominal aortic reconstruction.结缔组织病类型影响开放胸腹主动脉重建中移植物的分支通畅性。
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