Abstract

A 41-year-old patient with achondroplasia who had undergone surgery for congenital aortic stenosis >20 years ago presented with dyspnoea due to severe aortic stenosis. Computed tomography confirmed a small (16-mm) aortic annulus, thickened aortic valve leaflets without calcification and subaortic fibrous tissues. Intraoperatively, 3 non-calcific aortic leaflets were observed. Enlargement of the aortic root using a modified Manouguian technique for good exposure enabled the resection of subaortic tissues and replacement of the valve with a mechanical valve. The aortic root can be extremely small in patients with aortic stenosis and achondroplasia. The anatomy of the aortic root should be carefully assessed to enable appropriate surgical planning.

中文翻译：

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软骨发育不全性侏儒严重非钙化主动脉瓣狭窄罕见病例：手术考虑

摘要

一名 41 岁的软骨发育不全患者 > 20 年前因先天性主动脉瓣狭窄接受了手术，因严重主动脉瓣狭窄而出现呼吸困难。计算机断层扫描证实了一个小的（16 毫米）主动脉瓣环，主动脉瓣叶增厚，没有钙化和主动脉下纤维组织。术中观察到 3 个非钙化主动脉瓣。使用改良的 Manouguian 技术扩大主动脉根部以获得良好的暴露，从而能够切除主动脉下组织并用机械瓣膜置换瓣膜。主动脉瓣狭窄和软骨发育不全患者的主动脉根部可能非常小。应仔细评估主动脉根部的解剖结构，以制定适当的手术计划。