The aim is to investigate the value of novel MRI parameters for aortic hemodynamics and aortic motion and pressure waveforms in MFS patients as potential new biomarkers for guiding surgical and medical treatment of MFS, by analysing these parameters…
ID
Source
Brief title
Condition
- Cardiac and vascular disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
There are two main study parameters end points:
1. The difference in 3D aortic displacement expressed in mm, OSI
(dimensionless) and PWV (m/s) between MFS patient and healthy control aortas.
2. Change in 3D aortic displacement expressed in mm, OSI (dimensionless) and
PWV (m/s) between MFS patient aortas prior to compared to after PEARS procedure
or conventional aortic surgery.
Secondary outcome
1. Differences in advanced MRI parameters (displacement, OSI and PWV) between
MFS patients who have had aortic root replacement and MFS patients with a
native aorta.
2. Differences in advanced MRI parameters(displacement, OSI and PWV) between
high and low risk MFS patients based on aortic sinus diameter >4.5cm and <=4.5cm
3. Differences in advanced MRI parameters (displacement, OSI and PWV) between
MFS patients who have a HI FBN1 mutation or a DN FBN1 mutation.
4. Differences in advanced MRI parameters (displacement, OSI and PWV) between
genders in MFS patients.
5. Differences in advanced MRI parameters (displacement, OSI and PWV) between
MFS patients who use different cardiac medication.
6. The correlation between regional WSS and elastic fibre thickness in resected
aorta tissue of MFS patients.
7. The correlation between biomechanical tissue stiffness and elastic fibre
thickness
8. Differences in the extracellular matrix of cultured Marfan muscle cells
based on mutation type.
9. The correlation between pressure and flow waveform morphologies and aortic
root geometry in MFS patients with a native aortic root.
Background summary
Better understanding of aortic hemodynamics and motion in Marfan syndrome (MFS)
is required to ultimately improve indication criteria for surgical
intervention. In-house developed cardiac magnetic resonance imaging protocols
allow us to acquire advanced and detailed functional vascular parameters. The
Personalised External Aortic Root Support (PEARS) procedure is an emerging
alternative for early surgical intervention on the aorta root, with major
benefits, mainly concerning perioperative risks. To our knowledge evaluation of
advanced MRI parameters and pressure waveforms in MFS after PEARS procedure has
not previously been done.
Study objective
The aim is to investigate the value of novel MRI parameters for aortic
hemodynamics and aortic motion and pressure waveforms in MFS patients as
potential new biomarkers for guiding surgical and medical treatment of MFS, by
analysing these parameters in MFS patients in different disease stages (e.g.
native aorta, after conventional aorta surgery and after PEARS procedure) and
comparing the acquired values with healthy controls.
Study design
Observational design with a longitudinal design for a subgroup of patients.
Study burden and risks
Most procedures are part of standard clinical care. Added burden of current
study is the addition of study sequences to the MRI investigation, which will
result in an increase of scan time of twenty to thirty minutes and a subsequent
MRI sessions for a subgroup of patients after aortic surgery. The pressure
waveform analysis will take an additional 15 minutes.
Meibergdreef 9
Amsterdam 1105AZ
NL
Meibergdreef 9
Amsterdam 1105AZ
NL
Listed location countries
Age
Inclusion criteria
In order to be eligible to participate in this study, a subject must meet all
of the following criteria:
- Marfan Syndrome patients with a known Fibrilline-1 mutation
- Between 18-50 years of age
Inclusion criteria for participation in the surgery substudy:
- Marfan Syndrome patients with a known Fibrilline-1 mutation
- Planned elective thoracic aorta surgery (aortic (root) replacement, or PEARS
procedure)
Healthy controls:
- Between 18-50 years of age
Exclusion criteria
A potential subject who meets any of the following criteria (both patients and
healthy controls) will be excluded from participation in this study:
- Contraindication for MR imaging
- Mental retardation
- Pregnancy, or planned pregnancy during study period
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL82669.018.22 |