title: Surgical treatment of valvular and supravalvular aortic stenosis in homozygous familial hypercholesterolemia.
abstract: 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.
authors: Department of Cardiovascular Surgery, Shin-Koga Hospital, Tenjin-machi 120, Kurume, Fukuoka, 830-8577, Japan. trueno1977@yahoo.co.jp.
authors: Hisashi
authors: H
authors: Sato
authors: Department of Cardiovascular Surgery, Shin-Koga Hospital, Tenjin-machi 120, Kurume, Fukuoka, 830-8577, Japan.
authors: Masaru
authors: M
authors: Yoshikai
authors: Department of Cardiovascular Surgery, Shin-Koga Hospital, Tenjin-machi 120, Kurume, Fukuoka, 830-8577, Japan.
authors: Kazuyuki
authors: K
authors: Ikeda
authors: Department of Cardiovascular Surgery, Shin-Koga Hospital, Tenjin-machi 120, Kurume, Fukuoka, 830-8577, Japan.
authors: Yosuke
authors: Y
authors: Mukae
mesh_terms: D000818:Animals; D021921:Aortic Stenosis, Supravalvular / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006328:Cardiac Catheterization; D002417:Cattle; D004452:Echocardiography; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000150:complications*; D008077:Lipoproteins, LDL / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010496:Pericardium / Q000637:transplantation; D013524:Surgical Flaps; D014057:Tomography, X-Ray Computed
chemical_list: D008077:Lipoproteins, LDL
keywords: Aortic valve stenosis; Homozygous familial hypercholesterolemia; Supravalvular aortic stenosis