title: Endovascular treatment of type B dissection in patients with Marfan syndrome: mid-term outcomes and aortic remodeling.
abstract: OBJECTIVE
  To evaluate the mid-term outcomes, and the aortic remodeling in Marfan syndrome (MFS) patients with type B dissection that were treated with endovascular repair.
  
  
  BACKGROUND
  MFS is a relative contraindication to thoracic endovascular aortic repair (TEVAR). Mid-term aortic outcomes data in MFS after TEVAR are limited, and the occurrence of late events remains unclear.
  
  
  METHODS
  Of 89 patients that underwent TEVAR between September 2002 and February 2011, 10 patients with mid-term follow-up fulfilled the Ghent criteria for MFS and complicated type B dissection. High risk for open surgery was documented in 90%.
  
  
  RESULTS
  The mean age was 35.1 ± 9.4 years and all patients presented with acute aortic syndrome complicating a chronic type B dissection (DeBakey type IIIb). Five patients underwent a Bentall surgical procedure previous to endovascular repair, and in four patients initial TEVAR was followed by surgery of the ascending aorta. Treatment was limited to endovascular repair in only one patient. In-hospital mortality was 10%. At a mean follow-up of 59.6 ± 38.9 months, the cumulated mortality was of 20% and late mortality 11.1%. The rate of secondary endoleak was 44.4%, and late reintervention of 33.3%. Survival freedom from cardiovascular death at 8 years was 80.0%, and positive remodeling was documented in 37.5% of patients.
  
  
  CONCLUSIONS
  Our results suggest that TEVAR is feasible, safe, and associated with a high reintervention rate and reduced rate of positive aortic remodeling in patients with Marfan syndrome. Survival at 8 years was comparable to contemporary series of open repair.
authors.affiliation: Department of Interventional Cardiology, Instituto Nacional de Cardiología "Ignacio Chávez", Mexico City, Mexico.
authors.forename: Guering
authors.initials: G
authors.lastname: Eid-Lidt
authors.forename: Jorge
authors.initials: J
authors.lastname: Gaspar
authors.forename: Gabriela
authors.initials: G
authors.lastname: Meléndez-Ramírez
authors.forename: Jorge
authors.initials: J
authors.lastname: Cervantes S
authors.forename: Hector
authors.initials: H
authors.lastname: González-Pacheco
authors.forename: Félix
authors.initials: F
authors.lastname: Dámas de Los Santos
authors.forename: Aloha
authors.initials: A
authors.lastname: Meave-González
authors.forename: Samuel
authors.initials: S
authors.lastname: Ramírez Marroquín
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001027:Aortography / Q000379:methods; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000401:mortality; D002908:Chronic Disease; D004108:Dilatation, Pathologic; D057867:Endoleak / Q000209:etiology / Q000601:surgery; D057510:Endovascular Procedures / Q000009:adverse effects / Q000401:mortality; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D018810:Magnetic Resonance Angiography; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D012086:Reoperation; D012307:Risk Factors; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: Marfan syndrome; aortic dissection; endovascular aortic repair; stent grafts