title: Impaired biventricular deformation in Marfan syndrome: a strain and strain rate study in adult unoperated patients.
abstract: OBJECTIVE
  To investigate the presence of any regional myocardial deformation abnormalities in Marfan syndrome (MFS) and determine the benefits of using advanced echocardiography compared to conventional techniques.
  
  
  BACKGROUND
  Myocardial dysfunction in MFS may be caused by extracellular matrix remodeling thus, resulting in uniform reduced functionality. However, increased aortic stiffness may cause segmental ventricular abnormalities. Strain rate imaging (SRI) constitutes a validated technique to assess regional deformation in various clinical conditions. With this in mind, we aimed to investigate biventricular function in MFS using SRI.
  
  
  METHODS
  Forty-four MFS patients (mean age 30 ± 12 years, 26 men) and 49 controls without valvular disease were examined using SRI. Ejection fraction (EF) was calculated by the Simpson's biplane method. Biventricular deformation was assessed by measuring strain/strain rate. Strain values were divided by left ventricular (LV) end-diastolic volume to adjust LV deformation for geometry changes providing a strain index (SI). Aortic stiffness was evaluated using the β-stiffness index.
  
  
  RESULTS
  EF (%) was reduced in MFS patients (59 ± 5 vs 72 ± 4, P < 0.001), whereas β-stiffness was increased (P < 0.001). LV radial and LV and right ventricular (RV) long-axis strain values (%) were reduced in the patient group (70 ± 17 vs 93 ± 10; 19 ± 2 vs 25 ± 2; 30 ± 9 vs 36 ± 8, respectively, P < 0.001). Strain rate measurements were also reduced (P < 0.001). In a multiple regression analysis, MFS diagnosis was negatively associated with LV SI (-0.262 [-0.306, -0.219], P < 0.001). β-Stiffness was negatively associated with SI obtained from the septum, inferior and anterior walls. ROC analyses demonstrated that SRI, when compared with conventional echocardiography, had higher sensitivity and specificity in predicting biventricular dysfunction in MFS.
  
  
  CONCLUSIONS
  Our study showed a uniform reduction in biventricular deformation in MFS. These findings suggest that assessment of myocardial function using advanced echocardiographic techniques could be more accurate in MFS patient evaluation than conventional echocardiography alone.
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: Samir
authors.initials: S
authors.lastname: Saha
authors.forename: James C
authors.initials: JC
authors.lastname: Moggridge
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: 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: Bart H
authors.initials: BH
authors.lastname: Bijnens
authors.forename: Anne
authors.initials: A
authors.lastname: Child
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D003971:Diastole / Q000502:physiology; D004452:Echocardiography / Q000379:methods*; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D012372:ROC Curve; D015203:Reproducibility of Results; D013599:Systole / Q000502:physiology; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000503:physiopathology*