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European Journal of Echocardiography Advance Access originally published online on September 29, 2009
European Journal of Echocardiography 2009 10(8):947-955; doi:10.1093/ejechocard/jep110
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org

Biventricular and atrial diastolic function assessment using conventional echocardiography and tissue-Doppler imaging in adults with Marfan syndrome{dagger}

Anatoli Kiotsekoglou1, James C. Moggridge1, Bart H. Bijnens2, Venediktos Kapetanakis3, Francisco Alpendurada4, Michael J. Mullen4, Samir Saha5, 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 ICREA-Universitat Pompeu Fabra (CISTIB) and CIBER-BBN, Barcelona, Spain
3 MRC Biostatistics Unit, Institute of Public Health, Cambridge, UK
4 Royal Brompton and Harefield Hospital NHS Trust, Sydney Street, London SW3 6NP, UK
5 Thoracic and Vascular Division, Department of Clinical Physiology, Karolinska University Hospital at Huddinge, Stockholm 141 86, Sweden

Received 9 April 2009; accepted after revision 20 August 2009; online publish-ahead-of-print 29 September 2009.

* Corresponding author. Tel: +44 208 725 5248; fax: +44 208 725 2653. E-mail address: achild{at}sgul.ac.uk


  Abstract

Aims: Previous studies provided evidence about left ventricular systolic and diastolic dysfunction in adults with Marfan syndrome (MFS). However, in the literature, data on right ventricular and bi-atrial diastolic function are limited. We aimed to investigate whether, in the absence of significant valvular disease, diastolic dysfunction is present not only in both ventricles but also in the atrial cavities.

Methods and results: Seventy-two adult unoperated MFS patients and 73 controls without significant differences in age, sex, and body surface area from the patient group were studied using two-dimensional, pulsed, and colour-Doppler and tissue-Doppler imaging (TDI). Biventricular early filling measurements were significantly decreased in MFS patients when compared with controls (P < 0.001). Pulsed TDI early filling measurements obtained from five mitral annular regions and over the lateral tricuspid valve corner were significantly reduced in the patient group (P < 0.001). Indices reflecting atrial function at the reservoir, conduit and contractile phases were also significantly decreased in MFS patients (P < 0.001).

Conclusion: This study demonstrated significant biventricular diastolic and biatrial systolic and diastolic dysfunction in MFS patients. Our findings suggest that MFS affects diastolic function independently. Diastolic abnormalities could be attributed to fibrillin-1 deficiency and dysregulation of transforming growth factor-β activity in the cardiac extracellular matrix.

Keywords: Marfan syndrome; Fibrillin-1; Transforming growth factor beta; Ventricular diastolic function; Atrial diastolic function

{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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