The Marfan Trust is delighted to announce that it has had an important paper accepted for publication based on its ground breaking Aortic Irbesartan Marfan Study (AIMS). The study, co-funded by the Marfan Trust, the British Heart Foundation and the Marfan Association will be published in the Lancet journal before year end 2019 and is the culmination of a tremendous amount of work over eight years, by volunteer patients, physicians and nurses from 22 UK collaborating centres. Congratulations to all.
The study found that the use of the drug Irbesartan by children and young adults with Marfan syndrome could significantly improve their life expectancy.
Here is an extract from the paper:
Background: Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, may reduce aortic dilatation in Marfan syndrome which is associated with dissection and rupture.
Methods: Study participants with Marfan syndrome aged 6 to 40 years were all given 75mg open label irbesartan once daily, then randomised to 150mg (increased to 300mg as tolerated) or matching placebo in a multi-centre trial. Aortic diameter was measured by chocardiography at baseline and then annually. All images were analysed by a core laboratory blinded to treatment allocation. The primary endpoint was the rate of aortic root dilatation.
Findings: A total of 192 participants were randomised and followed for up to five years. Median age was 18 years, 52% were female, mean blood pressure was 110/65 mmHg and 56% were taking beta blockers. Mean baseline aortic root diameter was 34.4 mm. The mean rate of aortic root dilatation was 0.53 mm/year in the irbesartan group compared to 0.74 mm/year in the placebo group, difference in means -0.22 mm/year (95% confidence interval (CI) -0.41 to -0.02; p=0.030). The rate of change in aortic Z-score was also reduced by irbesartan (difference in means -0.10 per year; 95% CI -0.19 to -0.01; p=0.035). Systolic and diastolic blood pressure were reduced by irbesartan. Irbesartan was well tolerated with no observed differences in rates of serious adverse events.
Interpretation: Irbesartan is associated with a reduction in the rate of aortic dilatation in children and young adults with Marfan syndrome which could reduce the incidence of aortic complications.
Randomised trial of Irbesartan in Marfan syndrome: effects on aortic root dilatation (AIMS trial). Authors: Dr Michael Mullen MD FRCP, Dr Xu Yu Jin PhD FRCP, Dr Anne Child MD FRCP, Dr Graham Stuart MBBS, MSc, FRCP, Mr Matthew Dodd MSc, Dr José Antonio Aragon-Martin BSc PhD et. al.