Department of Cardiac Surgery, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.
Ann Thorac Surg. 2013 Feb;95(2):737-49. doi: 10.1016/j.athoracsur.2012.09.059. Epub 2012 Dec 28.
Marfan syndrome is the most frequently inherited disorder of connective tissue and is strongly associated with aortic dilatation, dissection, and rupture; in these patients, type B dissection occurs substantially. It is not known whether stent grafting, which is now frequently used in type B aortic dissection and descending thoracic aneurysms in non-Marfan patients, is a valuable option in Marfan patients, and reports from the literature are sparse and sporadic. We performed a systematic review of studies reporting the early and late results of endovascular stent grafting in Marfan patients with type B dissection in the attempt to quantify possible benefits or potential drawbacks of this approach in these usually very sick patients. Although associated with a low operative risk (1.9%), endovascular stent grafting in patients with Marfan syndrome carries a substantial risk of early and late complications, mainly endoleaks and surgical conversions, and of death at midterm follow-up. Because these complications are relatively more frequent in patients undergoing endovascular stent grafting for chronic dissections, these data suggest caution against the routine use of endovascular stent grafting in Marfan patients.
马凡综合征是最常见的结缔组织遗传性疾病，与主动脉扩张、夹层和破裂密切相关；在这些患者中，B 型夹层的发生率较高。目前尚不清楚支架移植术是否是马凡综合征患者的一种有价值的选择，因为在非马凡综合征患者中，这种方法常用于 B 型主动脉夹层和降胸主动脉瘤，而文献中的报道则很少且零星。我们对报道马凡综合征患者 B 型夹层血管内支架移植术早期和晚期结果的研究进行了系统回顾，试图量化这种方法在这些通常病情非常严重的患者中的可能益处或潜在缺点。尽管手术风险较低（1.9%），但马凡综合征患者的血管内支架移植术存在较高的早期和晚期并发症风险，主要是内漏和手术转换，以及中期随访时的死亡风险。由于这些并发症在接受慢性夹层血管内支架移植术的患者中更为常见，这些数据表明在马凡综合征患者中应谨慎使用血管内支架移植术。
