title: Assessment of aortic stiffness in marfan syndrome using two-dimensional and Doppler echocardiography.
abstract: BACKGROUND
  Extracellular matrix remodeling in the aortic wall results in increased aortic stiffness (AoS) in Marfan syndrome (MFS). Pulsed-wave velocity (PWV) constitutes the best indirect AoS measurement. We aimed to assess PWV in MFS patients using two-dimensional (2D) and Doppler echocardiography.
  
  
  METHODS
  Thirty-one MFS patients, (mean age 31 ± 14 years, 16 men) and 31 controls were examined. Blood flow was recorded in the aorta near the aortic valve and immediately after in the descending aorta with simultaneous electrocardiography. PWV was calculated by dividing the distance between the two sample volume positions (D) by the time difference (TD) between the intervals from the QRS start to the ascending and descending aortic flow onsets. B-stiffness was also measured.
  
  
  RESULTS
  TD (described in "Methods" section) and, aortic arch length were significantly increased in MFS patients, P < 0.001. Thus, PWV values were significantly higher in patients when compared with controls, 7.20 m/s (5.12, 9.43) versus 4.64 m/s (3.37, 6.24), P < 0.001. B-stiffness was also significantly increased in MFS patients; 5.15 (3.69, 7.65) versus 2.44 (1.82, 3.66), P < 0.001. Multiple regression analysis showed a positive association with MFS diagnosis and age, (P = 0.002 and 0.009, respectively). Reproducibility of PWV measurements was <5%.
  
  
  CONCLUSIONS
  AoS was significantly higher in MFS patients as expected. Our data demonstrated that PWV measurements can be performed, in the absence of serious musculoskeletal abnormalities in MFS adults, as part of a cardiac ultrasound scan. This technique can be helpful in diagnosis and management in MFS.
authors.affiliation: Department of Cardiac and Vascular Sciences, St. George's University of London, London, UK.
authors.forename: Anatoli
authors.initials: A
authors.lastname: Kiotsekoglou
authors.forename: James C
authors.initials: JC
authors.lastname: Moggridge
authors.forename: Samir K
authors.initials: SK
authors.lastname: Saha
authors.forename: Venediktos
authors.initials: V
authors.lastname: Kapetanakis
authors.forename: Malini
authors.initials: M
authors.lastname: Govindan
authors.forename: Francisco
authors.initials: F
authors.lastname: Alpendurada
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Mullen
authors.forename: John
authors.initials: J
authors.lastname: Camm
authors.forename: George R
authors.initials: GR
authors.lastname: Sutherland
authors.forename: Bart H
authors.initials: BH
authors.lastname: Bijnens
authors.forename: Anne H
authors.initials: AH
authors.lastname: Child
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001011:Aorta / Q000000981:diagnostic imaging* / Q000473:pathology*; D016022:Case-Control Studies; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging*; D008875:Middle Aged; D015588:Observer Variation; D055815:Young Adult