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瓦氏窦瘤样扩张——超越马方综合征:单中心经验及文献综述

Aneurysmal dilatation of the aortic sinuses of Valsalva -- beyond Marfan syndrome: a single centre experience and review of the literature.

作者信息

Caruana Maryanne, Sheppard Mary N, Li Wei

机构信息

Department of Cardiology, Mater Dei Hospital, Birkirkara Bypass, Birkirkara, MSD 2090, Malta,

出版信息

Front Med. 2014 Dec;8(4):419-26. doi: 10.1007/s11684-014-0383-6. Epub 2014 Dec 1.

Abstract

Aneurysmal dilatation of the aortic sinuses of Valsalva has been most extensively documented in the setting of aortopathies, particularly Marfan syndrome. On the other hand, there is limited data in the literature about congenital sinus of Valsalva aneurysms outside this context. For the purpose of this review, we carried out a literature search on aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome, and compared this with congenital sinus of Valsalva aneurysms, also including data from a case series from our institution. In conclusion, there are differences in management of aortic dilatation in Marfan syndrome and congenital sinus of Valsalva aneurysms. Though less well-recognised, congenital aneurysms are often associated with significant morbidity and mortality and timely intervention is necessary.

摘要

主动脉窦瘤样扩张在主动脉病变尤其是马方综合征的背景下已有最广泛的文献记载。另一方面,在这种背景之外,关于先天性主动脉窦瘤的文献数据有限。为了本次综述的目的,我们对马方综合征中主动脉窦瘤样扩张进行了文献检索,并将其与先天性主动脉窦瘤进行比较,还纳入了我们机构一个病例系列的数据。总之,马方综合征和先天性主动脉窦瘤在主动脉扩张的处理上存在差异。尽管先天性主动脉窦瘤的认知度较低,但它们常伴有显著的发病率和死亡率,及时干预是必要的。

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