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70 岁时诊断出非典型 Shone 综合征:表现为二尖瓣双孔、主动脉瓣二瓣化畸形和主动脉缩窄。

Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve, Bicuspid Aortic Valve, and Aortic Coarctation.

机构信息

Department of Cardiovascular Biology and Medicine, Juntendo University Graduate School of Medicine, Japan.

Department of Cardiovascular Biology and Medicine, Juntendo University Faculty of Medicine, Japan.

出版信息

Intern Med. 2022 May 1;61(9):1367-1370. doi: 10.2169/internalmedicine.8176-21. Epub 2021 Oct 19.

Abstract

Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.

摘要

非典型 Shone 综合征是一种罕见的先天性异常,涉及两个或三个心血管水平的左侧阻塞性病变。一位 70 岁的男性因劳力性呼吸困难被诊断为严重的主动脉瓣狭窄(AS)合并二叶式主动脉瓣,并发严重的主动脉缩窄(CoA)和双孔二尖瓣。他在一次手术中接受了 AS 和 CoA 的治疗。即使在老年发现二叶式主动脉瓣的情况下,也应仔细检查是否存在复杂的畸形。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7596/9152849/488ad2d959d3/1349-7235-61-1367-g001.jpg

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