title: Presentation and outcome of Marfan's syndrome patients with dissection and thoraco-abdominal aortic aneurysm.
abstract: BACKGROUND
  In Marfan's syndrome, there is a paucity of data regarding intervention criteria for surgery of the dissected thoraco-abdominal aorta.
  
  
  METHODS
  A retrospective analysis of 22 Marfan's patients with distal aortic dissection managed between September 1999 and April 2006 was performed. Serial diameters and linear expansion rates were calculated from imaging studies and the outcome of intervention was analysed.
  
  
  RESULTS
  There were 14/22 male patients (median age 38 years), and 18 had prior aortic surgery. Surgery was recommended in 20 patients and undertaken in 19 (1 died prior to operation). Of the operated patients, 2 presented with rupture, 2 with airway obstruction, 1 with intermittent paraplegia and 14 underwent planned surgery for increased expansion rate or pain. All patients had residual type A or chronic type B dissection. The median aortic dimension at surgery was 6.7 cm (interquartile range (IQR) 5.5-8.2). The preoperative mean expansion rate increased from 0.5 cm/year to 1.7 cm/year (p<0.001), prior to operation. Fifteen patients underwent Crawford Extent II, two underwent Extent I and two underwent Extent III repair. Profound hypothermia and CSF drainage was used in 16 and 18 patients, respectively. There was no early mortality, paraplegia or renal failure. At a median postoperative follow-up of 56 months (range 6-86), the survival of the operated cohort was 90%.
  
  
  CONCLUSIONS
  Thoraco-abdominal aortic aneurysm repair in Marfan's syndrome can be performed with good outcomes. Intervention should be based on size or accelerated expansion. Any role of endovascular management needs careful consideration.
authors.affiliation: Department of Cardiothoracic Surgery, University Hospital Birmingham NHS Trust, Queen Elizabeth Hospital, Birmingham B15 2TH, United Kingdom.
authors.forename: Maninder S
authors.initials: MS
authors.lastname: Kalkat
authors.forename: Ishtiaq
authors.initials: I
authors.lastname: Rahman
authors.forename: Kostas
authors.initials: K
authors.lastname: Kotidis
authors.forename: Ben
authors.initials: B
authors.lastname: Davies
authors.forename: Robert S
authors.initials: RS
authors.lastname: Bonser
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000150:complications / Q000473:pathology / Q000601:surgery*; D001011:Aorta / Q000601:surgery; D001014:Aortic Aneurysm / Q000150:complications / Q000473:pathology / Q000601:surgery*; D017544:Aortic Aneurysm, Abdominal / Q000150:complications / Q000473:pathology / Q000601:surgery; D017545:Aortic Aneurysm, Thoracic / Q000150:complications / Q000473:pathology / Q000601:surgery; D001019:Aortic Rupture / Q000150:complications / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000473:pathology / Q000601:surgery*; D008875:Middle Aged; D011183:Postoperative Complications; D012086:Reoperation; D012189:Retrospective Studies; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods