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洛伊斯-迪茨综合征中巨大升主动脉瘤的小儿Bentall手术

Pediatric Bentall procedure for giant ascending aortic aneurysm in Loeys-Dietz syndrome.

作者信息

Sicim Hüseyin, Ulucan Ali Ertan, Yurdakök Okan, Aydemir Numan Ali

机构信息

Department of Pediatric Cardiac Surgery, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, Istanbul, Turkey.

出版信息

Ann Pediatr Cardiol. 2023 May-Jun;16(3):223-225. doi: 10.4103/apc.apc_22_23. Epub 2023 Sep 8.

Abstract

Ascending aortic aneurysm is very rare in children, and is usually seen in patients with underlying connective tissue disorders such as Marfans and Ehler-Danlos syndrome. Loeys-Dietz syndrome (LDS) is less commonly seen as a cause of ascending aortic aneurysms in children. In this case report, we describe pediatric Bentall procedure, which we successfully performed to a child with LDS (Type I) with giant ascending aortic enlargement and significant aortic regurgitation.

摘要

升主动脉瘤在儿童中非常罕见,通常见于患有潜在结缔组织疾病的患者,如马凡综合征和埃勒斯-当洛综合征。洛伊-迪茨综合征(LDS)作为儿童升主动脉瘤的病因较少见。在本病例报告中,我们描述了小儿Bentall手术,我们成功地为一名患有LDS(I型)且升主动脉巨大扩张并伴有严重主动脉瓣反流的儿童实施了该手术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d776/10593284/119249e8ea0c/APC-16-223-g001.jpg

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