Stent graft placement of the thoracoabdominal aorta in a patient with Marfan syndrome.
Stent graft placement of the thoracoabdominal aorta in a patient with Marfan syndrome.
复制标题
马凡综合征患者胸腹主动脉支架植入术。
DOI：
10.1016/s0022-5223(02)73606-4
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发表时间：
2003
影响因子：
6
通讯作者：
M. Grabenwoger
中科院分区：
文献类型：
--
作者：
T. Fleck;D. Hutschala;H. Tschernich;E. Rieder;M. Czerny;E. Wolner;M. Grabenwoger
讨论已经进行了几项研究来确定马凡氏综合征患者的生存率和无事件生存率。他们表明，至少34%的马凡氏综合征患者在确诊后的前10年内会出现严重的心血管并发症，需要进行大的心脏手术。马凡氏综合征的主要心血管表现是影响主动脉根部和升主动脉，占这些患者总死亡率的82%。最近的报告强调了预防性主动脉根部置换术在这些患者中的重要性，与未接受手术干预的患者的34%相比，其10年生存率提高至97%。3在短时间间隔内对整个主动脉进行反复评估仍然是马凡氏综合征患者随访研究的主要内容。然而，在少数患者中，如本文所述，动脉瘤也发生在主动脉分支。3，4主动脉分支可能发生动脉瘤，如马凡氏综合征患者的锁骨下动脉或颈动脉，这强烈提示不仅要密切观察主动脉，而且要密切观察内脏和主动脉上分支。
DiscussionSeveral studies had been conducted to determine survival and event-free survival in patients with Marfan syndrome. 3, 4 They showed that at least 34% of patients with Marfan syndrome will have serious cardiovascular complications requiring major cardiac surgery in the first 10 years after diagnosis. The predominant cardiovascular manifestation of the Marfan syndrome affect the aortic root and ascending aorta counting for 82% overall mortality in these patients. Recent reports emphasized the importance of prophylactic replacement of the aortic root in these patients, which improves 10-year survival to 97% compared with 34% in patients who did not undergo surgical intervention. 3 Recurrent evaluation of the entire aorta in short intervals remains the mainstay of follow-up investigations in patients with Marfan syndrome. However, in a few patients, such as the one presented here, aneurysms also develop in aortic branches. 3, 4 The possibility of aneurysms developing in aortic branches, as in the subclavian or carotid arteries in patients with Marfan syndrome, strongly suggests the importance of close observation not only of the aorta but also of the visceral and supra-aortic branches.