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Intrinsic biventricular dysfunction in Marfan syndrome

Authors :
Jan J.J. Aalberts
Piet de Witte
Maarten Groenink
Janneke Timmermans
Maarten P. van den Berg
Arthur J. Scholte
Teodora Radonic
Aeilko H. Zwinderman
Barbara J.M. Mulder
ACS - Amsterdam Cardiovascular Sciences
Cardiology
Other departments
APH - Amsterdam Public Health
Epidemiology and Data Science
Radiology and Nuclear Medicine
Pathology
CCA - Cancer biology and immunology
CCA - Imaging and biomarkers
Faculteit der Geneeskunde
Cardiovascular Centre (CVC)
Source :
Heart (British Cardiac Society), 97(24), 2063-2068. BMJ Publishing Group, Heart, 97(24), 2063-2068. BMJ Publishing Group, De Witte, P, Aalberts, J J J, Radonic, T, Timmermans, J, Scholte, A J, Zwinderman, A H, Mulder, B J M, Groenink, M & Van Den Berg, M P 2011, ' Intrinsic biventricular dysfunction in Marfan syndrome ', Heart, vol. 97, no. 24, pp. 2063-2068 . https://doi.org/10.1136/heartjnl-2011-300169, Heart, 97(24), 2063-2068. BMJ PUBLISHING GROUP
Publication Year :
2011

Abstract

Background: Marfan syndrome (MFS) is an autosomal, dominantly inherited, connective tissue disorder usually caused by a mutation in the fibrillin-1 gene (FBN1). As fibrillin-1 is a component of the extracellular matrix of the myocardium, mutations in FBN1 may cause impairment of ventricular function. Furthermore, aortic elasticity is decreased in patients with MFS, which might also impair ventricular function. We assessed biventricular function and the influence of aortic elasticity in patients with MFS by means of cardiac MRI. Methods and results: Cardiac magnetic resonance was performed in 144 patients with MFS without significant valvular dysfunction, previous cardiac surgery or previous aortic surgery. Biventricular diastolic and systolic volumes were measured, and ejection fractions were calculated. Flow wave velocity, a measurable derivative of aortic elasticity, was measured between the ascending aorta and the bifurcation. When compared to healthy controls (n=19), left ventricular ejection fraction (LVEF) was impaired in patients with MFS (53%67% vs 57%±4%, p

Details

Language :
English
ISSN :
13556037
Database :
OpenAIRE
Journal :
Heart (British Cardiac Society), 97(24), 2063-2068. BMJ Publishing Group, Heart, 97(24), 2063-2068. BMJ Publishing Group, De Witte, P, Aalberts, J J J, Radonic, T, Timmermans, J, Scholte, A J, Zwinderman, A H, Mulder, B J M, Groenink, M & Van Den Berg, M P 2011, ' Intrinsic biventricular dysfunction in Marfan syndrome ', Heart, vol. 97, no. 24, pp. 2063-2068 . https://doi.org/10.1136/heartjnl-2011-300169, Heart, 97(24), 2063-2068. BMJ PUBLISHING GROUP
Accession number :
edsair.doi.dedup.....85ad8c11eba1a0ae10a8ded7a044199e
Full Text :
https://doi.org/10.1136/heartjnl-2011-300169