Marfan Syndrome
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: Entry criteria: ? Clinical diagnosis of Marfan syndrome (Ghent criteria) ? Aged 16-50 years; either gender On no treatment for Marfan syndrome, or on beta-blocker or other monotherapy only Are the trial subjects under 18? yes Number of subjects for this age range: F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range F.1.3 Elderly (>=65 years) no F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: Exclusion criteria: ? Previous aortic dissection or aortic surgery ? Severe aortic or mitral regurgitation; or cardiac disease unrelated to Marfan Syndrome ? Pregnancy, or of child-bearing age if at risk of pregnancy ? Aortic diameter at sinotubular junction = 5.0 cm ? Contraindications to specific drug treatment: e.g. asthma (?-blockers), or renal dysfunction defined as serum creatinine >150 µmol/l (ACE inhibitor)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: In patients with Marfan syndrome: ? oral beta-blockade (atenolol) does not reduce central aortic stiffness or pressure ? ACE inhibition (perindopril) reduces central arterial pressure and improves aortic compliance ? a non-dihydropyridine calcium antagonist (verapamil) reduces rate of rise of arterial pressure, aortic stiffness, and central arterial pressure ? genotype affects the response to treatment ;Secondary Objective: ; Primary end point(s): The predefined primary end-points are beta index and aortic wave speed (indices of large artery stiffness), percentage augmentation (as a measure of central arterial pressure), and the amplitude of the forward compression wave (an index of myocardial contractile function). | — |
Countries
United Kingdom