title: Increased aortic stiffness in prepubertal girls with Turner syndrome.
abstract: Aortic dilation and dissection contribute highly to the increased mortality of Turner syndrome (TS) but the exact pathophysiology is not completely understood.
  
  
  
  Prospective case - control study.
  
  
  
  15 prepubertal TS girls (median age 10.64, IQ 8.31-11.04) with a tricuspid (TAV, n=9) or a bicuspid (BAV, n=6) aortic valve, and 31 sex-, age-, and height-matched healthy controls underwent a cardiac and vascular ultrasound to evaluate aortic dimensions and elastic properties of the aortic wall.
  
  
  
  TS BAV had significantly larger ascending aortic diameters than controls for absolute diameter, 22.2±5.1mm vs. 18.6±1.9mm (p=0.014) and z-score 1.7±2.1 vs. 0.1±0.7 (p=0.008). Distensibility of the ascending aorta was lower in the TS than in controls (40.2×10-3kPa-1, IQ 31.3-56.2 vs. 62.9×10-3kPa-1, IQ 55.5-76.5, p=0.003), both for TS TAV (p=0.014) and BAV (p=0.005). Stiffness index was higher in TS than in controls (5.26, IQ 3.34-5.26 vs. 3.23, IQ 2.55-3.24, p=0.005), both for TS TAV (p=0.028) and TS BAV (p=0.006). Pulse wave velocity was not different between groups. There was no correlation between stiffness and z-score of the ascending aortic diameter.
  
  
  
  In prepubertal TS girls, stiffness of the ascending aorta is increased in patients with a BAV and TAV while dilation of the ascending aorta is more frequent in BAV. This suggests an intrinsic aortic wall abnormality making all TS patients at increased risk for severe aortic complications although the risk is the highest for TS with BAV.
authors.affiliation: Pediatric Cardiology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, Ghent, Belgium. Electronic address: katya.degroote@uzgent.be.
authors.forename: Katya
authors.initials: K
authors.lastname: De Groote
authors.affiliation: Cardiovascular Radiology, Department of Radiology and Turner Clinic, Ghent University Hospital, Ghent, Belgium.
authors.forename: Daniel
authors.initials: D
authors.lastname: Devos
authors.affiliation: Department of Public Health, Ghent University, Ghent, Belgium; Pediatric Cardiology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Koen
authors.initials: K
authors.lastname: Van Herck
authors.affiliation: Pediatric Cardiology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Daniel
authors.initials: D
authors.lastname: De Wolf
authors.affiliation: Pediatric Endocrinology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Saskia
authors.initials: S
authors.lastname: Van der Straaten
authors.affiliation: Department of Cardiology, Ghent University Hospital and Ghent University, Ghent University Hospital, Ghent, Belgium.
authors.forename: Ernst
authors.initials: E
authors.lastname: Rietzschel
authors.affiliation: Pediatric Nephrology, Department of Pediatrics and Safepedrug, Ghent University Hospital, Ghent, Belgium.
authors.forename: Ann
authors.initials: A
authors.lastname: Raes
authors.affiliation: Pediatric Cardiology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Kristof
authors.initials: K
authors.lastname: Vandekerckhove
authors.affiliation: Pediatric Cardiology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Joseph
authors.initials: J
authors.lastname: Panzer
authors.affiliation: Pediatric Cardiology, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium.
authors.forename: Hans
authors.initials: H
authors.lastname: De Wilde
authors.affiliation: Pediatric Endocrinology, Department of Pediatrics and Turner Clinic, Ghent University Hospital, Ghent, Belgium.
authors.forename: Jean
authors.initials: J
authors.lastname: De Schepper
mesh_terms: D001011:Aorta / Q000473:pathology / Q000503:physiopathology; D001018:Aortic Diseases / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D001021:Aortic Valve / Q000473:pathology / Q000503:physiopathology; D016022:Case-Control Studies; D002648:Child; D004108:Dilatation, Pathologic; D005260:Female; D006349:Heart Valve Diseases / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D011446:Prospective Studies; D063177:Pulse Wave Analysis; D014424:Turner Syndrome / Q000150:complications / Q000401:mortality / Q000503:physiopathology*; D059289:Vascular Stiffness / Q000502:physiology*
keywords: Aortic dilation; Aortic elastic properties; Aortic rupture; Childhood; Echocardiography