Back to Search
Start Over
Intrinsic biventricular dysfunction in Marfan syndrome
- 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
- Subjects :
- Marfan syndrome
Male
Aorta, Thoracic
030204 cardiovascular system & hematology
Severity of Illness Index
030218 nuclear medicine & medical imaging
Marfan Syndrome
0302 clinical medicine
AXIS SYSTOLIC FUNCTION
Ventricular Dysfunction
Pulse wave velocity
Ejection fraction
NORMAL-SIZED AORTA
DISTENSIBILITY
Prognosis
Cardiac surgery
Echocardiography
Cardiology
Disease Progression
cardiovascular system
Female
Cardiology and Cardiovascular Medicine
Cardiac function curve
Adult
musculoskeletal diseases
medicine.medical_specialty
DIMENSIONS
congenital, hereditary, and neonatal diseases and abnormalities
animal structures
Heart Ventricles
Diastole
Magnetic Resonance Imaging, Cine
03 medical and health sciences
CARDIAC-FUNCTION
LEFT-VENTRICULAR FUNCTION
Internal medicine
medicine.artery
Ascending aorta
medicine
Humans
cardiovascular diseases
BETA-BLOCKER THERAPY
Aorta
business.industry
Stroke Volume
SIGNIFICANT VALVULAR REGURGITATION
ADULTS
medicine.disease
Elasticity
PULSE-WAVE VELOCITY
business
Subjects
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