title: The risk for type B aortic dissection in Marfan syndrome.
abstract: BACKGROUND
  Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome.
  
  
  OBJECTIVE
  The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome.
  
  
  METHODS
  Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome.
  
  
  RESULTS
  Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome (mean age 36 ± 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery (hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter ≥27 mm (hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections (hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030).
  
  
  CONCLUSIONS
  Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections.
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands.
authors.forename: Alexander W
authors.initials: AW
authors.lastname: den Hartog
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands.
authors.forename: Romy
authors.initials: R
authors.lastname: Franken
authors.affiliation: Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Aeilko H
authors.initials: AH
authors.lastname: Zwinderman
authors.affiliation: Department of Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Janneke
authors.initials: J
authors.lastname: Timmermans
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
authors.forename: Arthur J
authors.initials: AJ
authors.lastname: Scholte
authors.affiliation: Department of Cardiology, University Medical Center, Groningen, the Netherlands.
authors.forename: Maarten P
authors.initials: MP
authors.lastname: van den Berg
authors.affiliation: Department of Cardiology, University Medical Center, Groningen, the Netherlands; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Vivian
authors.initials: V
authors.lastname: de Waard
authors.affiliation: Department of Clinical Genetics, VU University Medical Center, Amsterdam, the Netherlands.
authors.forename: Gerard
authors.initials: G
authors.lastname: Pals
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands.
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Radiology, Academic Medical Center, Amsterdam, the Netherlands. Electronic address: m.groenink@amc.uva.nl.
authors.forename: Maarten
authors.initials: M
authors.lastname: Groenink
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000145:classification / Q000209:etiology* / Q000517:prevention & control; D057911:Angiotensin Receptor Antagonists / Q000627:therapeutic use; D001013:Aorta, Thoracic / Q000473:pathology / Q000601:surgery; D017545:Aortic Aneurysm, Thoracic / Q000145:classification / Q000209:etiology* / Q000517:prevention & control; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D015999:Multivariate Analysis; D018570:Risk Assessment; D014057:Tomography, X-Ray Computed
chemical_list: D057911:Angiotensin Receptor Antagonists
keywords: Marfan syndrome; aortic diameter; aortic distensibility; aortic dissection; aortic root replacement; distal aorta; losartan