The purpose of this study is to determine whether new magnetic resonance imaging techniques can be used as a biomarker of aortic disease severity in patients with Marfan syndrome.
Study Type
OBSERVATIONAL
Enrollment
148
3D CINE and 4 dimensional (4D) flow MRI sequence
Radboud University Medical Center
Nijmegen, Gelderland, Netherlands
Leiden University Medical Center
Leiden, North Holland, Netherlands
Amsterdam UMC, location AMC
Amsterdam, Netherlands
University Medical Center Groningen
Groningen, Netherlands
Difference in 3D aortic displacement in millimeters between Marfan patients (MFS) and healthy volunteers.
aortic displacement in the thoracic aorta. normal values unknown, but single paper: Marfan patients range: 1.77 to 10.03 millimeters, volunteers 7.34 ± 1.69 millimeters. lower is worse.
Time frame: Baseline
Difference in pulse wave velocity, in meters per second between Marfan patients and healthy volunteers.
pulse wave velocity expressed in m/s in the thoracic aorta, normal values age dependent: but range +/- 4 - 30 m/s, higher is worse
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between Marfan patients and healthy volunteers
oscillatory shear index (dimensionless) in the thoracic aorta, range between 0 - 0.5 (No normal values defined), higher is worse
Time frame: Baseline
Change in aortic displacement expressed in millimeters , between MFS patient aortas prior to compared to after PEARS procedure or conventional aortic surgery.
aortic displacement expressed in millimeters in the thoracic aorta. Pre-post change unknown, however operated 3.60 +/- 1.0 millimeter versus native aorta 7.01 +/- 3.09 millimeters. Lower is worse
Time frame: Change from baseline to 6 months after surgery
Change in aortic pulse wave velocity (meters/second), between MFS patient aortas prior to compared to after PEARS procedure or conventional aortic surgery.
pulse wave velocity in the thoracic aorta expressed in meters/second. Higher is worse. normal values for change unknown.
Time frame: Change from baseline to 6 months after surgery
Change in oscillatory shear index (dimensionless) between MFS patient aortas prior to compared to after PEARS procedure or conventional aortic surgery.
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oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse range between 0 -5.
Time frame: Change from baseline to 6 months after surgery
Difference in 3D aortic displacement expressed in mm between Marfan patients with and without a native aorta
aortic displacement in the thoracic aorta, lower is worse. operated 3.60 +/- 1.0 millimeter versus native aorta 7.01 +/- 3.09 millimeters
Time frame: Baseline
Difference in 3D aortic displacement expressed in mm between Marfan patients with aortic root diameter >4.5cm versus <4.5 cm
aortic displacement in the thoracic aorta, lower is worse. Normal values unknown.
Time frame: Baseline
Difference in 3D aortic displacement expressed in mm between Marfan patients with a haploinsufficient fibrillin 1 mutation versus dominant negative mutation
aortic displacement in the thoracic aorta. Lower is worse. Normal values unknown.
Time frame: Baseline
Difference in 3D aortic displacement expressed in mm between male versus female Marfan patients
aortic displacement in the thoracic aorta,Lower is worse. Normal values unknown.
Time frame: Baseline
Difference in 3D aortic displacement expressed in mm between Marfan patients based on antihypertensive medication used
aortic displacement in the thoracic aorta, Lower is worse. Normal values unknown.
Time frame: Baseline
Difference in pulse wave velocity, expressed in meters per second between Marfan patients with and without a native aorta
pulse wave velocity expressed in m/s in the thoracic aorta, Lower is worse. Normal values unknown. Single study in general aneurysm population: pre surgery: 18.0 +/-4.0 meters per second post surgery 20.6 +/- 6.0 meters per second. higher is worse
Time frame: Baseline
Difference in pulse wave velocity, expressed in m/s between Marfan patients with an aortic root diameter >4.5 cm versus <4.5 cm
pulse wave velocity expressed in m/s in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in pulse wave velocity, expressed in m/s between Marfan patients with a haploinsufficient fibrillin-1 mutation versus a dominant negative mutation
pulse wave velocity expressed in m/s in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in pulse wave velocity, expressed in m/s between male and female Marfan patients
pulse wave velocity expressed in m/s in the thoracic aorta Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in pulse wave velocity, expressed in m/s between Marfan patients based on antihypertensive medication used
pulse wave velocity expressed in m/s in the thoracic aorta
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between Marfan patients with and without a native aorta
oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between Marfan patients with aortic root diameter >4.5cm versus <4.5 cm
oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between Marfan patients with a haploinsufficient fibrillin 1 mutation versus dominant negative mutation
oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between male versus female Marfan patients
oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
Difference in oscillatory shear index (dimensionless) expressed in m/s between Marfan patients based on antihypertensive medication used
oscillatory shear index (dimensionless) in the thoracic aorta. Higher is worse. Normal values unknown.
Time frame: Baseline
The correlation between regional wall shear stress and elastic fibre thickness in resected aorta tissue of MFS patients.
Correlation of locally MRI measured wall shear stress and histologic findings in the thoracic aorta. Neither is worse. Normal values dependent on location of measurement, exploratory outcome. .
Time frame: Baseline MRI with aortic surgery tissue