title: Overview of current surgical strategies for aortic disease in patients with Marfan syndrome.
abstract: Marfan syndrome is a heritable, systemic disorder of the connective tissue with a high penetrance, named after Dr. Antoine Marfan. The most clinically important manifestations of this syndrome are cardiovascular pathologies which cause life-threatening events, such as acute aortic dissections, aortic rupture and regurgitation of the aortic valve or other artrioventricular valves leading to heart failure. These events play important roles in the life expectancy of patients with this disorder, especially prior to the development of effective surgical approaches for proximal ascending aortic disease. To prevent such catastrophic aortic events, a lower threshold has been recommended for prophylactic interventions on the aortic root. After prophylactic root replacement, disease in the aorta beyond the root and distal to the arch remains a cause for concern. Multiple surgeries are required throughout a patient's lifetime that can be problematic due to distal lesions complicated by dissection. Many controversies in surgical strategies remain, such as endovascular repair, to manage such complex cases. This review examines the trends in surgical strategies for the treatment of cardiovascular disease in patients with Marfan syndrome, and current perspectives in this field.
authors.affiliation: Division of Cardiovascular Surgery, Department Surgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Miyahara
authors.affiliation: Division of Cardiovascular Surgery, Department Surgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan. yokita@med.kobe-u.ac.jp.
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Okita
mesh_terms: D000784:Aortic Dissection / Q000601:surgery; D001012:Aorta, Abdominal / Q000601:surgery; D001013:Aorta, Thoracic / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery; D001018:Aortic Diseases / Q000517:prevention & control / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D013504:Cardiovascular Surgical Procedures / Q000379:methods* / Q000639:trends*; D057510:Endovascular Procedures; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008382:Marfan Syndrome / Q000601:surgery*; D008943:Mitral Valve / Q000601:surgery
keywords: Aortic dissection; Aortic root replacement; Marfan syndrome