Surgical treatment of valvular and supravalvular aortic stenosis in homozygous familial hypercholesterolemia

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• 作者：Hisashi Sato ; Masaru Yoshikai…
• 关键词：Homozygous familial hypercholesterolemia ; Aortic valve stenosis ; Supravalvular aortic stenosis
• 刊名：General Thoracic and Cardiovascular Surgery
• 出版年：2016
• 出版时间：February 2016
• 年：2016
• 卷：64
• 期：2
• 页码：98-100
• 全文大小：749 KB
• 参考文献：1.Sprecher DL, Schaefer EJ, Kent KM, Gregg RE, Zech LA, Hoeg JM, et al. Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients. Am J Cardiol. 1984;54:20–30.PubMed CrossRef
  2.Yasuda T, Kawasuji M, Sakakibara N, Watanabe Y. Aortic valve replacement for the calcified aorta in homozygous familial hypercholesterolemia. Eur J Cardiothorac Surg. 2000;18:249–50.PubMed CrossRef
  3.Saito S, Usui A, Akita T, Ueda Y. Surgery for calcific aortic stenosis in homozygous familial hypercholesterolemia. Eur J Cardiothorac Surg. 2006;29:114–6.PubMed CrossRef
  
• 作者单位：Hisashi Sato (1)
  Masaru Yoshikai (1)
  Kazuyuki Ikeda (1)
  Yosuke Mukae (1)
  
  1. Department of Cardiovascular Surgery, Shin-Koga Hospital, Tenjin-machi 120, Kurume, Fukuoka, 830-8577, Japan
  
• 刊物主题：Thoracic Surgery; Cardiac Surgery; Cardiology; Surgical Oncology;
• 出版者：Springer Japan
• ISSN：1863-6713

文摘

A 61-year-old male with homozygous familial hypercholesterolemia presented with dyspnea and syncope. He had been treated with low-density lipoprotein apheresis for 26 years. Echocardiography and computed tomography showed severe valvular and supravalvular aortic stenosis. Computed tomography and cardiac catheterization revealed a severely calcified narrowed aortic root and an occlusion in the proximal right coronary artery. During surgery, the ascending aorta was replaced under deep hypothermic circulatory arrest without aortic cross-clamping. After that, the aortic root from the annulus to the sino-tubular junction was enlarged with a two-ply bovine pericardial patch. An aortic valve replacement with a 17 mm mechanical valve and coronary artery bypass grafting to the right coronary artery were performed. The patient recovered from the surgery without any cerebrovascular complications. Keywords Homozygous familial hypercholesterolemia Aortic valve stenosis Supravalvular aortic stenosis