title: Endovascular management of chronic aortic dissection in patients with Marfan syndrome.
abstract: BACKGROUND
  Marfan syndrome patients are prone to aortic dilatation, dissection, and rupture. Success of aortic root replacement has generated a cohort of patients surviving longer and presenting with distal aortic dissection and enlargement. Thoracic endovascular stent-graft repair (TEVR) is being increasingly utilized to exclude aneurysms resulting from chronic aortic dissection. This report explores the role of TEVR in Marfan patients with this pathology.
  
  
  METHODS
  Review of a prospectively maintained database identified seven patients with Marfan syndrome offered endovascular repair of aneurysmal chronic aortic dissection. All patients had previous aortic root repair. Talent or Valiant (Medtronic Vascular, Santa Rosa, Calif) aortic stent-grafts were used to occlude the dissection entry tear and cover the thoracic aorta. Electronic data, case notes, and radiological surveillance were analyzed.
  
  
  RESULTS
  Seven consecutive patients (six male; mean age, 45.9 +/- 10 years, range, 29 to 63) underwent successful thoracic stent-graft deployment. Mean aortic aneurysmal diameter was 63.4mm (+/-11.2) with six of seven dissections extending to the aortic bifurcation. No perioperative neurological events occurred. Thirty-day mortality was 1/7 (14%) due to congestive cardiac failure. At median 16 month follow-up, two of six cases (33%) required intervention for endoleak. Aortic false lumen thrombosis (FLT) occurred in 5/6 (83%) cases and partial FLT occurred in 1/6 (17%). All thoracic aortas continued to dilate during follow-up. Crude median aortic growth rate was 7.2 mm/year (range, 3.5 to 19 mm).
  
  
  CONCLUSIONS
  TEVR in Marfan syndrome patients with chronic aortic dissection is technically feasible. However, post intervention surveillance confirms that the aorta continues to dilate despite graft deployment and false lumen thrombosis. Endovascular repair may offer a viable option in patients who have contraindications to open surgery, but longer follow up of more patients is required to define the place of this therapy.
authors.affiliation: St. George's Vascular Institute, St. James' Wing, St. George's Hospital, London, UK. inordon@sgul.ac.uk
authors.forename: Ian M
authors.initials: IM
authors.lastname: Nordon
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Hinchliffe
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Holt
authors.forename: Rob
authors.initials: R
authors.lastname: Morgan
authors.forename: Marjan
authors.initials: M
authors.lastname: Jahangiri
authors.forename: Ian M
authors.initials: IM
authors.lastname: Loftus
authors.forename: Matt M
authors.initials: MM
authors.lastname: Thompson
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001014:Aortic Aneurysm / Q000000981:diagnostic imaging / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001027:Aortography; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation* / Q000401:mortality; D002908:Chronic Disease; D018450:Disease Progression; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D008875:Middle Aged; D011475:Prosthesis Failure; D012086:Reoperation; D012189:Retrospective Studies; D015607:Stents; D013997:Time Factors; D016896:Treatment Outcome