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Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients.
- Source :
-
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance [J Cardiovasc Magn Reson] 2017 Mar 17; Vol. 19 (1), pp. 30. Date of Electronic Publication: 2017 Mar 17. - Publication Year :
- 2017
-
Abstract
- Background: Blood flow dynamics make it possible to better understand the development of aortopathy and cardiovascular events in patients with Marfan syndrome (MFS). Aortic 3D blood flow characteristics were investigated in relation to aortic geometry in children and adolescents with MFS.<br />Methods: Twenty-five MFS patients (age 15.6 ± 4.0 years; 11 females) and 21 healthy controls (age 16.0 ± 2.6 years; 12 females) underwent magnetic resonance angiography and 4D flow CMR for assessment of thoracic aortic size and 3D blood flow velocities. Data analysis included calculation of aortic diameter and BSA-indexed aortic dimensions (Z-score) along the thoracic aorta, 3D mean systolic wall shear stress (WSS <subscript>mean</subscript> ) in ten aortic segments and assessment of aortic blood flow patterns.<br />Results: Aortic root (root), ascending (AAo) and descending (DAo) aortic size was significantly larger in MFS patients than healthy controls (Root Z-score: 3.56 ± 1.45 vs 0.49 ± 0.78, p < 0.001; AAo Z-score 0.21 ± 0.95 vs -0.54 ± 0.64, p = 0.004; proximal DAo Z-score 2.02 ± 1.60 vs 0.56 ± 0.66, p < 0.001). A regional variation in prevalence and severity of flow patterns (vortex and helix flow patterns) was observed, with the aortic root and the proximal DAo (pDAo) being more frequently affected in MFS. MFS patients had significantly reduced WSS <subscript>mean</subscript> in the proximal AAo (pAAo) outer segment (0.65 ± 0.12 vs. 0.73 ± 0.14 Pa, p = 0.029) and pDAo inner segment (0.74 ± 0.17 vs. 0.87 ± 0.21 Pa, p = 0.021), as well as higher WSS <subscript>mean</subscript> in the inner segment of the distal AAo (0.94 ± 0.14 vs. 0.84 ± 0.15 Pa, p = 0.036) compared to healthy subjects. An inverse relationship existed between pDAo WSS <subscript>mean</subscript> and both pDAo diameter (R = -0.53, p < 0.001) and % diameter change along the pDAo segment (R = -0.64, p < 0.001).<br />Conclusions: MFS children and young adults have altered aortic flow patterns and differences in aortic WSS that were most pronounced in the pAAo and pDAo, segments where aortic dissection or rupture often originate. The presence of vortex flow patterns and abnormal WSS correlated with regional size of the pDAo and are potentially valuable additional markers of disease severity.
- Subjects :
- Adolescent
Age Factors
Aorta, Thoracic physiopathology
Blood Flow Velocity
Cardiovascular Diseases etiology
Cardiovascular Diseases physiopathology
Case-Control Studies
Child
Female
Humans
Male
Marfan Syndrome diagnosis
Predictive Value of Tests
Regional Blood Flow
Stress, Mechanical
Time Factors
Aorta, Thoracic diagnostic imaging
Cardiovascular Diseases diagnostic imaging
Hemodynamics
Image Interpretation, Computer-Assisted methods
Imaging, Three-Dimensional methods
Magnetic Resonance Imaging, Cine methods
Marfan Syndrome complications
Perfusion Imaging methods
Subjects
Details
- Language :
- English
- ISSN :
- 1532-429X
- Volume :
- 19
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance
- Publication Type :
- Academic Journal
- Accession number :
- 28302143
- Full Text :
- https://doi.org/10.1186/s12968-017-0345-7