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Title: Early impairment of left ventricular long-axis systolic function demonstrated by reduced atrioventricular plane displacement in patients with Marfan syndrome
Authors: Kiotsekoglou, Anatoli ×
Bajpai, Abhay
Bijnens, Bart
Kapetanakis, Venedictos
Athanassopoulos, George
Moggridge, James C
Mullen, Mike J
Nassiri, Dariush K
Camm, John
Sutherland, George R
Child, Anne H #
Issue Date: Sep-2008
Publisher: W.B. Saunders Co.
Series Title: European Journal of Echocardiography vol:9 issue:5 pages:605-613
Abstract: AIMS: 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). METHODS AND 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). CONCLUSION: 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.
URI: 
ISSN: 1525-2167
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Cardiovascular Imaging and Dynamics
× corresponding author
# (joint) last author

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