Marfan Syndrome
Conditions
Interventions
Administration of Metoprolol Losartan and a combination of both.
Sponsors
Amsterdam UMC
Eligibility
Age
18 Years to 64 Years
Inclusion criteria
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 Healthy controls: - Between 18-50 years of age
Exclusion criteria
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 Additional exclusion criteria for the medication sub study: - History of aorta surgery - More than 50mg daily Metoprolol usage at baseline - Contra-indication for Metoprolol and/or Losartan - Contra-indication for cardiopulmonary exercise testing (table 1) - Patients who use antihypertensive medication for the indication hypertension. - Patients with a baseline systolic blood pressure >140 mmHg and/or diastolic >90 mmHg. Additional exclusion criteria healthy controls: - History of aorta disease
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| There are three main study parameters end points 1. Any statistically significant differences in advanced MRI parameters between MFS patients and healthy controls. 2. Any statistically significant differences in advanced MRI parameters between MFS patients prior to compared to after PEARS procedure. 3. Any statistically significant differences in advanced MRI parameters within MFS patients using either ARB, BB or a combination of both | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Differences in advanced MRI parameters between MFS patients who have had aortic root replacement and MFS patients with a native aorta. 2. Differences in advanced MRI parameters between MFS patients who have had aortic root replacement and MFS patients with a native aorta. 3. Differences in advanced MRI parameters between high and low risk MFS patients based on aortic sinus diameter >4.5cm and *4.5cm 4. Differences in advanced MRI parameters between MFS patients who have a HI FBN1 mutation or a DN FBN1 mutation. 5. Differences in advanced MRI parameters between genders in MFS patients. 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. Differences in exercise capacity, quantified as peak oxygen consumption (peak VO2), within MFS patients using either ARB or BB. 10. Differences in quality of life and habitual physical activity within MFS patients using either ARB or BB. | — |
Countries
Netherlands
Outcome results
None listed