title: Impaired right ventricular systolic function demonstrated by reduced atrioventricular plane displacement in adults with Marfan syndrome.
abstract: OBJECTIVE
  The right ventricle (RV) ejects the same volume of blood at the same rate as the left ventricle (LV). Mild LV dysfunction has been demonstrated in Marfan syndrome (MFS). However, little attention has been paid to the functioning of the RV. The aim of this study was to assess RV function in unoperated adult MFS patients.
  
  
  RESULTS
  In 66 unoperated (15-58 years) MFS patients and 61 controls, rate of pressure rise (dp/dt) in RV, and tricuspid annular motion (TAM) were studied using conventional echocardiography and tissue Doppler imaging (TDI). When compared with controls, MFS patients showed impaired RV systolic function as expressed by a reduced dp/dt, TAM obtained by M-mode echocardiography, and peak TDI systolic velocities at the basal lateral wall (745.36+/-37.85 vs. 1103.30+/-27.30 mmHg, P<0.001; 2.2+/-0.05 vs. 2.5+/-0.05 cm, P<0.001; and 0.13+/-0.002 vs. 0.16+/-0.002 m/s, P<0.001, respectively).
  
  
  CONCLUSIONS
  This study demonstrated a primary impairment of RV systolic function in MFS. This is the first study to report RV dysfunction in MFS. Such data could prove valuable during the peri-operative and long-term medical management of MFS patients.
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: George R
authors.initials: GR
authors.lastname: Sutherland
authors.forename: James C
authors.initials: JC
authors.lastname: Moggridge
authors.forename: Venedictos
authors.initials: V
authors.lastname: Kapetanakis
authors.forename: Abhay
authors.initials: A
authors.lastname: Bajpai
authors.forename: Nicholas
authors.initials: N
authors.lastname: Bunce
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Mullen
authors.forename: George
authors.initials: G
authors.lastname: Louridas
authors.forename: Dariush K
authors.initials: DK
authors.lastname: Nassiri
authors.forename: John
authors.initials: J
authors.lastname: Camm
authors.forename: Anne H
authors.initials: AH
authors.lastname: Child
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D001283:Atrioventricular Node / Q000000981:diagnostic imaging / Q000473:pathology*; D001783:Blood Flow Velocity; D016022:Case-Control Studies; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000503:physiopathology*; D015201:Meta-Analysis as Topic; D008875:Middle Aged; D013599:Systole; D014261:Tricuspid Valve / Q000473:pathology*; D018608:Ultrasonography, Doppler; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016278:Ventricular Function, Right; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists