Hinojosa Carlos A, Anaya-Ayala Javier E, Laparra-Escareno Hugo, Lizola Rene, Torres-Machorro Adriana
Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Vasc Specialist Int. 2017 Sep;33(3):112-116. doi: 10.5758/vsi.2017.33.3.112. Epub 2017 Sep 30.
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.
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。