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European Journal of Echocardiography Advance Access published online on February 22, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen003
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Published on behalf of the European Society of Cardiography. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Early impairment of left ventricular long-axis systolic function demonstrated by reduced atrioventricular plane displacement in patients with Marfan syndrome{dagger}

Anatoli Kiotsekoglou1, Abhay Bajpai1, Bart H. Bijnens1, Venedictos Kapetanakis1, George Athanassopoulos2, James C. Moggridge1, Mike J. Mullen3, Dariush K. Nassiri1, John Camm1, George R. Sutherland1 and Anne H. Child1,*

1 Department of Cardiac and Vascular Sciences, St George's University of London, Cranmer Terrace, London SW17 0RE, UK
2 Onassis Cardiac Surgery Centre, 176 74 Kallithea, Athens, Greece
3 Royal Brompton and Harefield Hospital, NHS Trust, London SW3 6NP, UK

Received 20 July 2007; accepted after revision 23 October 2007.

* Corresponding author. Tel: + 44 208 7255248; fax: + 44 208 7252653. E-mail address: achild{at}sgul.ac.uk


  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-β 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.

Keywords: Marfan syndrome; Left ventricular systolic function; Atrioventricular plane displacement; Long-axis function; Tissue Doppler imaging

{dagger} This study was performed at St George's University of London and St George's Hospital NHS Trust, London, UK.


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A Kiotsekoglou, G R Sutherland, J C Moggridge, D K Nassiri, A J Camm, and A H Child
The unravelling of primary myocardial impairment in Marfan syndrome by modern echocardiography
Heart, October 1, 2009; 95(19): 1561 - 1566.
[Abstract] [Full Text] [PDF]


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