title: [Surgical treatment of Marfan syndrome; analysis of the patients required multiple surgical interventions].
abstract: Without treatment, the life expectancy of patients with Marfan syndrome is reduced by the associated cardiovascular abnormalities. In this study, we reviewed our experience of the patients with Marfan syndrome who required multiple surgical interventions to identify the optimal treatment for these patients. Between January 1986 and December 2000, 44 patients with Marfan syndrome were operated on at Shizuoka City Hospital (SCH). Among them, 10 patients (22.7%) underwent multiple surgical interventions. There were 5 male and 5 female patients with a mean age of 40.6 +/- 16.1 years at the initial surgery. Only one patient was operated on at another hospital for his first, second, and third operations. His fourth operation was carried out at SCH. The remaining 9 patients underwent a total of 14 additional surgical procedures at SCH. Computed tomography (CT) scans were taken every 6 months postoperatively, and aortic diameter greater than 60 mm was considered as the indication for the additional surgery. There were no early death and one late death. The causes of additional surgery were enlargement of true aneurysm in 6, enlargement of residual dissection in 4, new dissection in 4, false aneurysm at the coronary anastomosis of Bentall procedure in 1. In 9 patients, both ascending and descending aorta were replaced. Among these 9 patients, only 3 patients underwent total arch replacement, and remaining 6 patients had their arch left in place with or without dissection. Our current strategy of the treatment of Marfan patients with acute type A dissection is total arch replacement with an elephant trunk at the initial emergent surgery.
authors.affiliation: Department of Cardiovascular Surgery, Shizuoka City Hospital, Shizuoka, Japan.
authors.forename: F
authors.initials: F
authors.lastname: Yamazaki
authors.forename: M
authors.initials: M
authors.lastname: Shimamoto
authors.forename: S
authors.initials: S
authors.lastname: Fujita
authors.forename: M
authors.initials: M
authors.lastname: Nakai
authors.forename: A
authors.initials: A
authors.lastname: Aoyama
authors.forename: F
authors.initials: F
authors.lastname: Chen
authors.forename: T
authors.initials: T
authors.lastname: Nakata
authors.forename: T
authors.initials: T
authors.lastname: Yamada
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000601:surgery; D001013:Aorta, Thoracic / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000601:surgery*; D008875:Middle Aged; D011379:Prognosis; D011859:Radiography; D012086:Reoperation; D013997:Time Factors