title: Marked aortic valve stenosis progression after receiving long-term aggressive cholesterol-lowering therapy using low-density lipoprotein apheresis in a patient with familial hypercholesterolemia.
abstract: In 1982, a 49-year-old Japanese woman had been referred to our hospital for further investigation of her hypercholesterolemia. She was diagnosed as heterozygous familial hypercholesterolemia, because of Achilles tendon xanthoma and a family history of primary hypercholesterolemia. Three years later, she had chest pain on effort and angina pectoris was diagnosed by coronary angiography. At that time, she underwent coronary artery bypass grafting surgery with 2 saphenous vein grafts (SVG). Because more aggressive cholesterol-lowering therapy was needed for secondary prevention of coronary artery disease (CAD), weekly low-density lipoprotein (LDL) apheresis was started postoperatively, combined with drug therapy. Since 1986, her serum total cholesterol levels before and after LDL apheresis remained approximately 200 mg/dl and 90 mg/dl, respectively. Although her coronary sclerosis, including the SVG, did not progress appreciably for a period of 20 years, stenotic changes of the aortic valve developed rapidly at age 70, leading to aortic valve replacement surgery in 2005 at age 72. These findings suggest that careful attention to the progression of aortic valve stenosis is needed for extreme hypercholesterolemic patients even under optimal cholesterol-lowering therapy for the secondary prevention of CAD.
authors.affiliation: Division of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Tsuchida
authors.forename: Masa-aki
authors.initials: MA
authors.lastname: Kawashiri
authors.forename: Hayato
authors.initials: H
authors.lastname: Tada
authors.forename: Mutsuko
authors.initials: M
authors.lastname: Takata
authors.forename: Atsushi
authors.initials: A
authors.lastname: Nohara
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Ino
authors.forename: Akihiro
authors.initials: A
authors.lastname: Inazu
authors.forename: Junji
authors.initials: J
authors.lastname: Kobayashi
authors.forename: Junji
authors.initials: J
authors.lastname: Koizumi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mabuchi
authors.forename: Masakazu
authors.initials: M
authors.lastname: Yamagishi
mesh_terms: D000787:Angina Pectoris / Q000209:etiology; D000924:Anticholesteremic Agents / Q000627:therapeutic use; D001024:Aortic Valve Stenosis / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D001781:Blood Component Removal; D002784:Cholesterol / Q000097:blood*; D002792:Cholestyramine Resin / Q000627:therapeutic use; D017023:Coronary Angiography; D001026:Coronary Artery Bypass; D003324:Coronary Artery Disease / Q000097:blood / Q000000981:diagnostic imaging / Q000209:etiology / Q000517:prevention & control* / Q000601:surgery; D018450:Disease Progression; D004452:Echocardiography; D005260:Female; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000097:blood / Q000150:complications / Q000628:therapy*; D008077:Lipoproteins, LDL / Q000097:blood*; D008875:Middle Aged; D055502:Secondary Prevention / Q000379:methods*
chemical_list: D000924:Anticholesteremic Agents; D008077:Lipoproteins, LDL; D002792:Cholestyramine Resin; D002784:Cholesterol