title: Patch annulo-aortoplasty in an adult patient with congenital supravalvular aortic stenosis and a small aortic annulus.
abstract: A 39-year-old woman with familial homozygous hypercholesterolemia had supravalvular and valvular aortic stenosis. Modified Nick's procedure and aortic valve replacement was performed to relieve both the supravalvular and annular stenoses. At surgery, the ascending aorta was found to be narrowing at the level of the sinotubular junction, which was compatible with congenital supravalvular aortic stenosis. Histological examination of the aortic cusps showed sclerotic change due to hypercholesterolemia. These findings indicated that familial homozygous hypercholesterolemia caused valvular aortic stenosis and exacerbated congenital supravalvular aortic stenosis.
authors.affiliation: Department of Cardiovascular Surgery, Himeji Cardiovascular Center, 520 Saisho-ko, Himeji, Hyogo, Japan. naotofrcs@gmail.com
authors.forename: Naoto
authors.initials: N
authors.lastname: Morimoto
authors.forename: Keisuke
authors.initials: K
authors.lastname: Morimoto
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Morimoto
authors.forename: Akiko
authors.initials: A
authors.lastname: Tanaka
authors.forename: Toshihito
authors.initials: T
authors.lastname: Sakamoto
authors.forename: Kenji
authors.initials: K
authors.lastname: Okada
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Okita
mesh_terms: D000328:Adult; D021921:Aortic Stenosis, Supravalvular / Q000151:congenital / Q000175:diagnosis / Q000601:surgery*; D001021:Aortic Valve / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D001781:Blood Component Removal; D058385:Cardiac Valve Annuloplasty; D017548:Echocardiography, Transesophageal; D005260:Female; D006720:Homozygote; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000150:complications* / Q000235:genetics / Q000628:therapy; D008077:Lipoproteins, LDL / Q000097:blood; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D008077:Lipoproteins, LDL