title: Bentall operation, total aortic replacement and mitral valve replacement for a young adult with Marfan syndrome: a case of three-staged operation.
abstract: In Marfan syndrome, the most common cardiovascular abnormalities are dilatation of the aorta and aortic valve regurgitation in adult patients. Mitral valve dysfunction is the most common cause of morbidity and mortality in infants and children with Marfan syndrome, and is not frequently operated on in adult Marfan patients who undergo surgery for diseases of the aortic root and total aorta. This report describes a successfully three-staged operation for a 24 year-old man with Marfan syndrome who underwent an emergent Bentall operation and aortic arch replacement, total aortic replacement and mitral valve replacement over 2 years. Mitral valve regurgitation was mild but increased after the second operation. The graft was tightly adhesive and invasive to the sternum. Endoscopic view was helpful to avoid graft damage at resternotomy. The postoperative course was uneventful in each operation. Microscopic examination of the mitral valve leaflets showed abnormal increase of mucopolysaccharides, and disruption and fragmentation of elastic fibers.
authors.affiliation: Second Department of Surgery, Yamagata University School of Medicine, 2-2-2 Iida-nishi, Yamagata 990-9585, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Inui
authors.forename: Y
authors.initials: Y
authors.lastname: Shimazaki
authors.forename: T
authors.initials: T
authors.lastname: Watanabe
authors.forename: S
authors.initials: S
authors.lastname: Kuraoka
authors.forename: T
authors.initials: T
authors.lastname: Minowa
authors.forename: M
authors.initials: M
authors.lastname: Miura
authors.forename: S
authors.initials: S
authors.lastname: Oshikiri
authors.forename: H
authors.initials: H
authors.lastname: Toyama
mesh_terms: D000328:Adult; D001011:Aorta / Q000002:abnormalities / Q000601:surgery*; D001027:Aortography; D019917:Blood Vessel Prosthesis Implantation; D004452:Echocardiography; D005500:Follow-Up Studies; D019918:Heart Valve Prosthesis Implantation / Q000379:methods; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D008943:Mitral Valve / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000601:surgery*; D008944:Mitral Valve Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D014057:Tomography, X-Ray Computed