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European Journal of Echocardiography Advance Access originally published online on September 18, 2008
European Journal of Echocardiography 2009 10(2):295-302; doi:10.1093/ejechocard/jen239
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org.

Impaired right ventricular systolic function demonstrated by reduced atrioventricular plane displacement in adults with Marfan syndrome

Anatoli Kiotsekoglou1, George R. Sutherland1, James C. Moggridge1, Venedictos Kapetanakis1, Abhay Bajpai1, Nicholas Bunce1, Michael J. Mullen2, George Louridas3, Dariush K. Nassiri1, John Camm1 and Anne H. Child1,*

1 Department of Cardiac and Vascular Sciences, St George’s, University of London, Cranmer Terrace, London SW17 0RE, UK
2 Royal Brompton and Harefield Hospital NHS Trust, Sydney Street, London SW3 6NP, UK
3 Department of Cardiology, AHEPA Hospital, Aristotle University of Thessaloniki, First Kiriakidi Street, Thessaloniki 546 36, Greece

Received 27 May 2008; accepted after revision 25 August 2008; online publish-ahead-of-print 18 September 2008.

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


  Abstract

Aims: 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.

Methods and 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).

Conclusion: 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.

Keywords: Marfan syndrome; Right ventricular function; Systolic long-axis function; Tissue Doppler imaging


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