Marfan Syndrome can cause the wall of the main blood vessel in the heart, known as the aorta, to expand. Without treatment, the aorta can expand too much and eventually it can rupture, leading to life threatening bleeding and potential death. The expansion of the aorta is known as aortic dilatation, and it is this complication we are investigating.
Current treatments to slow the rate of aortic dilatation include beta blockers, however these do not stop the aortic wall from expanding completely and eventually surgery maybe required to replace the base of the aorta, known as the aortic root and valve. Sometimes people with Marfan Syndrome cannot tolerate beta blockers because they have side effects such as dizziness and tiredness, and people with asthma cannot take them as well.
In this study we wish to investigate whether another drug known as Irbesartan can successfully reduce aortic dilatation effectively and safely in patients with Marfan Syndrome. Irbesartan is from a class of drugs known as Angiotensin II receptor antagonists which are currently used to reduce high blood pressure. Small pilot studies have indicated that they may well be better at reducing the rate of aortic dilatation in Marfan Syndrome, however this has not been properly tested in a controlled clinical trial.
The main purpose of this study is to evaluate how effective Irbesartan is for treating patients with Marfan Syndrome in reducing the rate of aortic dilatation. The study will also look at how safe the treatment is and how well it is tolerated. The treatment being given in this study is an additional treatment, which will be added to therapy currently being provided. Patients enrolled in this trial will not be expected to stop taking current medication.
