Saito Shunei, Usui Akihiko, Akita Toshiaki, Ueda Yuichi
Department of Cardiothoracic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya, Aichi 466-8550, Japan.
Eur J Cardiothorac Surg. 2006 Jan;29(1):114-6. doi: 10.1016/j.ejcts.2005.11.001. Epub 2005 Dec 6.
A 35-year-old female with homozygous familial hyperlipidemia (IIa) was referred to our hospital for an operation against supravalvular and valvular aortic stenosis. She had been treated with low-density lipoprotein apheresis for 20 years, and total cholesterol ranged between 200 and 400 mg/dl under this treatment. She had undergone percutaneous coronary intervention for ostial stenosis of the right coronary artery three times since the age of 19. Unenhanced three-dimensional computed tomography showed supravalvular stenosis, funnelling and heavily calcified aorta. An operation was performed under deep hypothermic circulatory arrest without aortic cross clamping. After the ascending aorta had been replaced with a one-branched vascular graft, arterial perfusion was resumed. The stenosed ascending aorta was resected at the sinotubular junction. Because the aortic root was still extremely small, the noncoronary sinus and the commissure between left and right coronary cusp were incised, and the aortic root was enlarged with linguiform vascular-graft patches. A 21-mm mechanical valve was implanted. The postoperative course was uneventful.
一名35岁的纯合子家族性高脂血症（IIa型）女性因针对瓣上和瓣膜性主动脉狭窄的手术被转诊至我院。她接受低密度脂蛋白单采治疗已有20年，在此治疗下总胆固醇在200至400mg/dl之间。自19岁起，她因右冠状动脉开口狭窄已接受过三次经皮冠状动脉介入治疗。非增强三维计算机断层扫描显示瓣上狭窄、漏斗样改变以及主动脉重度钙化。在深低温停循环且未进行主动脉阻断的情况下实施了手术。用单分支血管移植物替换升主动脉后，恢复动脉灌注。在窦管交界处以切除狭窄的升主动脉。由于主动脉根部仍然极小，切开无冠窦以及左右冠状动脉瓣叶之间的交界，并用舌状血管移植物补片扩大主动脉根部。植入了一枚21mm的机械瓣膜。术后病程平稳。
