title: MRI-assessed regional pulse wave velocity for predicting absence of regional aorta luminal growth in marfan syndrome.
abstract: BACKGROUND
  In patients with Marfan syndrome (MFS), increased aortic wall stiffening may lead to progressive aortic dilatation. Aortic Pulse Wave Velocity (PWV), a marker of wall stiffness can be assessed regionally, using in-plane multi-directional velocity-encoded MRI. This study examined the diagnostic accuracy of regional PWV for prediction of regional aortic luminal growth during 2-year follow-up in MFS patients.
  
  
  METHODS
  In twenty-one MFS patients (mean age 36 ± 15 years, 11 male) regional PWV and aortic luminal areas were assessed by 1.5 T MRI. At 2-year follow-up, the incidence of luminal growth, defined as mean luminal diameter increase >2mm was determined for five aortic segments (S1, ascending aorta; S2, aortic arch; S3, thoracic descending aorta, S4, supra-renal and S5, infra-renal abdominal aorta). Regional PWV at baseline was considered increased when exceeding age-related normal PWV (healthy volunteers (n=26; mean age 30 ± 10 years, 15 male)) by two standard-errors. Sensitivity and specificity of regional PWV-testing for prediction of regional luminal growth were determined.
  
  
  RESULTS
  Regional PWV at baseline was increased in 17 out of 102 segments (17%). Significant luminal growth at follow-up was reported in 14 segments (14%). The specificity of regional PWV-testing was ≥ 78% for all aortic segments, sensitivity was ≤ 33%.
  
  
  CONCLUSIONS
  Regional PWV was significantly increased in MFS patients as compared to healthy volunteers within similar age range, in all aortic segments except the ascending aorta. Furthermore, regional PWV-assessment has moderate to high specificity for predicting absence of regional aortic luminal growth for all aortic segments in MFS patients.
authors.affiliation: Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands. e.s.j.kroner@lumc.nl
authors.forename: Eleanore S J
authors.initials: ES
authors.lastname: Kröner
authors.forename: Arthur J H A
authors.initials: AJ
authors.lastname: Scholte
authors.forename: Patrick J H
authors.initials: PJ
authors.lastname: de Koning
authors.forename: Pieter J
authors.initials: PJ
authors.lastname: van den Boogaard
authors.forename: Lucia J M
authors.initials: LJ
authors.lastname: Kroft
authors.forename: Rob J
authors.initials: RJ
authors.lastname: van der Geest
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Hilhorst-Hofstee
authors.forename: Hildo J
authors.initials: HJ
authors.lastname: Lamb
authors.forename: Hans-Marc J
authors.initials: HM
authors.lastname: Siebelink
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
authors.forename: Maarten
authors.initials: M
authors.lastname: Groenink
authors.forename: Teodora
authors.initials: T
authors.lastname: Radonic
authors.forename: Ernst E
authors.initials: EE
authors.lastname: van der Wall
authors.forename: Albert
authors.initials: A
authors.lastname: de Roos
authors.forename: Johan H C
authors.initials: JH
authors.lastname: Reiber
authors.forename: Jos J M
authors.initials: JJ
authors.lastname: Westenberg
mesh_terms: D000328:Adult; D001011:Aorta / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D008875:Middle Aged; D011237:Predictive Value of Tests; D063177:Pulse Wave Analysis / Q000379:methods*; D016037:Single-Blind Method; D059289:Vascular Stiffness / Q000502:physiology*; D055815:Young Adult