title: Proximal aortic stiffening in Turner patients may be present before dilation can be detected: a segmental functional MRI study.
abstract: BACKGROUND
  To study segmental structural and functional aortic properties in Turner syndrome (TS) patients. Aortic abnormalities contribute to increased morbidity and mortality of women with Turner syndrome. Cardiovascular magnetic resonance (CMR) allows segmental study of aortic elastic properties.
  
  
  METHODS
  We performed Pulse Wave Velocity (PWV) and distensibility measurements using CMR of the thoracic and abdominal aorta in 55 TS-patients, aged 13-59y, and in a control population (n = 38;12-58y). We investigated the contribution of TS on aortic stiffness in our entire cohort, in bicuspid (BAV) versus tricuspid (TAV) aortic valve-morphology subgroups, and in the younger and older subgroups.
  
  
  RESULTS
  Differences in aortic properties were only seen at the most proximal aortic level. BAV Turner patients had significantly higher PWV, compared to TAV Turner (p = 0.014), who in turn had significantly higher PWV compared to controls (p = 0.010). BAV Turner patients had significantly larger ascending aortic (AA) luminal area and lower AA distensibility compared to both controls (all p < 0.01) and TAV Turner patients. TAV Turner had similar AA luminal areas and AA distensibility compared to Controls. Functional changes are present in younger and older Turner subjects, whereas ascending aortic dilation is prominent in older Turner patients. Clinically relevant dilatation (TAV and BAV) was associated with reduced distensibility.
  
  
  CONCLUSIONS
  Aortic stiffening and dilation in TS affects the proximal aorta, and is more pronounced, although not exclusively, in BAV TS patients. Functional abnormalities are present at an early age, suggesting an aortic wall disease inherent to the TS. Whether this increased stiffness at young age can predict later dilatation needs to be studied longitudinally.
authors.affiliation: Department of Radiology, MRI (-1K12), Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium. daniel.devos@uzgent.be.
authors.forename: Daniel G H
authors.initials: DG
authors.lastname: Devos
authors.affiliation: Pediatric Cardiology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Katya
authors.initials: K
authors.lastname: De Groote
authors.affiliation: Telecommunications and Information Processing, TELIN-IPI-iMinds, Faculty of Engineering and Architecture, Ghent University, Sint-Pietersnieuwstraat 41, 9000, Ghent, Belgium.
authors.forename: Danilo
authors.initials: D
authors.lastname: Babin
authors.affiliation: Department of Cardiology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Laurent
authors.initials: L
authors.lastname: Demulier
authors.affiliation: Department of Cardiology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Yves
authors.initials: Y
authors.lastname: Taeymans
authors.affiliation: Department of Radiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, The Netherlands.
authors.forename: Jos J
authors.initials: JJ
authors.lastname: Westenberg
authors.affiliation: Heymans Institute of Pharmacology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Luc
authors.initials: L
authors.lastname: Van Bortel
authors.affiliation: IBiTech-bioMMeda, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Patrick
authors.initials: P
authors.lastname: Segers
authors.affiliation: Department of Radiology, MRI (-1K12), Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Eric
authors.initials: E
authors.lastname: Achten
authors.affiliation: Pediatric Endocrinology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Jean
authors.initials: J
authors.lastname: De Schepper
authors.affiliation: Department of Cardiology, Ghent University Hospital, De Pintelaan 185, B-9000, Gent, Belgium.
authors.forename: Ernst
authors.initials: E
authors.lastname: Rietzschel
mesh_terms: D000293:Adolescent; D000328:Adult; D001012:Aorta, Abdominal / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging* / Q000503:physiopathology; D001018:Aortic Diseases / Q000000981:diagnostic imaging* / Q000209:etiology / Q000503:physiopathology; D001021:Aortic Valve / Q000002:abnormalities; D000082882:Bicuspid Aortic Valve Disease; D016022:Case-Control Studies; D002648:Child; D004108:Dilatation, Pathologic; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications / Q000175:diagnosis; D006801:Humans; D007090:Image Interpretation, Computer-Assisted; D019028:Magnetic Resonance Imaging, Cine; D008875:Middle Aged; D011237:Predictive Value of Tests; D011446:Prospective Studies; D063177:Pulse Wave Analysis; D012307:Risk Factors; D014424:Turner Syndrome / Q000150:complications* / Q000175:diagnosis; D059289:Vascular Stiffness; D055815:Young Adult