title: Early impairment of left ventricular long-axis systolic function demonstrated by reduced atrioventricular plane displacement in patients with Marfan syndrome.
abstract: OBJECTIVE
  Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the fibrillin-1 (FBN1) gene. It has been observed that FBN1 deficient mice have reduced left ventricular (LV) systolic function which is correlated to increased transforming growth factor-beta activity. This study aimed to ascertain LV functional abnormalities in MFS patients using M-mode and tissue Doppler imaging (TDI).
  
  
  RESULTS
  In 66 (15-58 years) MFS patients and 61 normal controls, ejection fraction (EF) was evaluated by Simpson's biplane method. Atrioventricular plane displacement (AVPD) obtained from five mitral annular regions was also assessed using M-mode and TDI techniques. To overcome limitations associated with conventional M-mode echocardiography, anatomical and colour anatomical M-mode were also utilized. Ejection fraction was significantly reduced in MFS patients when compared to controls (66.3 +/- 0.74 vs. 71.9 +/- 0.56, P < 0.001), although it was within the normal range. M-mode and TDI AVPD measurements obtained from lateral, septal, inferior, anterior and posterior mitral annular regions were also significantly reduced in MFS patients in comparison to controls (P <0.001, for all measurements).
  
  
  CONCLUSIONS
  Left ventricular long-axis systolic function is significantly reduced in MFS patients. This data suggests that LV function should be monitored in MFS and appropriate treatment applied if necessary.
authors.affiliation: Department of Cardiac and Vascular Sciences, St George's University of London, Cranmer Terrace, London SW17 0RE, UK.
authors.forename: Anatoli
authors.initials: A
authors.lastname: Kiotsekoglou
authors.forename: Abhay
authors.initials: A
authors.lastname: Bajpai
authors.forename: Bart H
authors.initials: BH
authors.lastname: Bijnens
authors.forename: Venedictos
authors.initials: V
authors.lastname: Kapetanakis
authors.forename: George
authors.initials: G
authors.lastname: Athanassopoulos
authors.forename: James C
authors.initials: JC
authors.lastname: Moggridge
authors.forename: Mike J
authors.initials: MJ
authors.lastname: Mullen
authors.forename: Dariush K
authors.initials: DK
authors.lastname: Nassiri
authors.forename: John
authors.initials: J
authors.lastname: Camm
authors.forename: George R
authors.initials: GR
authors.lastname: Sutherland
authors.forename: Anne H
authors.initials: AH
authors.lastname: Child
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001283:Atrioventricular Node / Q000000981:diagnostic imaging / Q000503:physiopathology*; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008875:Middle Aged; D013318:Stroke Volume; D013599:Systole; D013997:Time Factors; D014463:Ultrasonography; D055815:Young Adult