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Updated: Mar 6, 2026
Three-Dimensional Printing of a Complex Aortic Anomaly
Published on: November 1, 2018
Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients
Roel L F van der Palen1,2, Alex J Barker3, Emilie Bollache3
1Department of Radiology, Feinberg School of Medicine, Northwestern University , Chicago, IL, USA. r.vanderpalen@lumc.nl.
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.
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 (WSSmean) in ten aortic segments and assessment of aortic blood flow patterns.
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 WSSmean 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 WSSmean 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 WSSmean and both pDAo diameter (R = -0.53, p < 0.001) and % diameter change along the pDAo segment (R = -0.64, p < 0.001).
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.
Insights
Children and young adults with Marfan syndrome (MFS) exhibit altered aortic blood flow patterns and wall shear stress (WSS). These changes, particularly in the proximal ascending aorta (pAAo) and proximal descending aorta (pDAo), correlate with aortic size and may indicate disease severity.
Area of Science:
- Cardiovascular imaging and biomechanics
- Pediatric cardiology and genetics
Background:
- Marfan syndrome (MFS) is associated with aortopathy and cardiovascular events.
- Understanding blood flow dynamics in MFS is crucial for predicting disease progression.
Purpose of the Study:
- To investigate 3D blood flow characteristics in relation to aortic geometry in children and adolescents with MFS.
- To identify potential markers of disease severity in MFS patients.
Main Methods:
- 25 MFS patients and 21 healthy controls underwent 4D flow cardiovascular magnetic resonance (CMR).
- Thoracic aortic size, 3D blood flow velocities, and mean systolic wall shear stress (WSSmean) were assessed.
- Aortic dimensions (Z-score) and flow patterns (vortex, helix) were analyzed.
Main Results:
- MFS patients showed significantly larger aortic dimensions compared to controls.
- Altered flow patterns (vortex, helix) were more prevalent in the aortic root and proximal descending aorta (pDAo) in MFS patients.
- Reduced WSSmean was observed in the proximal ascending aorta (pAAo) outer segment and pDAo inner segment of MFS patients.
Conclusions:
- MFS children and young adults have distinct aortic flow patterns and WSS differences, most notable in the pAAo and pDAo.
- Vortex flow and abnormal WSS correlate with pDAo size, suggesting their utility as disease severity markers.
- These findings highlight the importance of advanced imaging in managing MFS-related aortopathy.
