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马凡综合征患者慢性主动脉夹层伴广泛动脉瘤样退变继发主动脉及双侧髂总动脉的序贯杂交修复术

Sequential Hybrid Repair of Aorta and Bilateral Common Iliac Arteries Secondary to Chronic Aortic Dissection with Extensive Aneurysmal Degeneration in a Marfan Patient.

作者信息

Hinojosa Carlos A, Anaya-Ayala Javier E, Laparra-Escareno Hugo, Lizola Rene, Torres-Machorro Adriana

机构信息

Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

出版信息

Vasc Specialist Int. 2017 Sep;33(3):112-116. doi: 10.5758/vsi.2017.33.3.112. Epub 2017 Sep 30.

Abstract

Marfan syndrome is a connective tissue disorder associated with aortic dissection, aneurysmal degeneration and rupture. These cardiovascular complications represent the main cause of mortality, therefore repair is indicated. We present a 35-year-old woman who experienced acute onset of chest pain. Her imaging revealed a chronic DeBakey type I dissection with aortic root dilation and descending thoracic aneurysmal degeneration. She underwent a Bentall procedure and endovascular exclusion of the descending thoracic aortic aneurysm. She was closely followed and 2 years later a computed tomography angiography (CTA) revealed the aneurysmal degeneration of the thoracoabominal aorta and bilateral iliac arteries. The patient underwent a composite reconstruction using multi-visceral branched and bifurcated Dacron grafts. At 5 years from her last surgery, a CTA revealed no new dissection or further aneurysmal degenerations. Aortic disease in Marfan patients is a complex clinical problem that may lead to secondary or tertiary aortic reconstructions; close follow-up is mandatory.

摘要

马凡综合征是一种与主动脉夹层、动脉瘤样变性和破裂相关的结缔组织疾病。这些心血管并发症是主要的死亡原因,因此需要进行修复。我们报告一名35岁女性,她突发胸痛。其影像学检查显示为慢性德巴基I型夹层,伴有主动脉根部扩张和降胸段动脉瘤样变性。她接受了Bentall手术及降胸段主动脉瘤的血管腔内隔绝术。对她进行了密切随访,2年后计算机断层血管造影(CTA)显示胸腹主动脉和双侧髂动脉出现动脉瘤样变性。该患者接受了使用多脏器分支和分叉涤纶移植物的复合重建手术。在她最后一次手术后5年,CTA显示没有新的夹层或进一步的动脉瘤样变性。马凡综合征患者的主动脉疾病是一个复杂的临床问题,可能需要进行二次或三次主动脉重建;必须进行密切随访。

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