BACKGROUND: Recent studies have demonstrated that blockade of the angiotensin II type 1 receptor with losartan decreases aortic damage in an animal model of Marfan syndrome (a KI mouse model with a pathogenic mutation in the gene coding for fibrillin-1). AIMS: To demonstrate a beneficial effect of losartan on aortic dilatation when added to optimal therapy in patients with Marfan syndrome. METHODS: This is a multicentre, randomized, placebo-controlled, double-blind, clinical trial with a 2-year inclusion period and a 3-year follow-up period. Aortic root diameter will be measured using two-dimensional echocardiography. Secondary endpoints will include incidence of aortic dissection, aortic root surgery, death, quality of life, tolerance and compliance with treatments. We aim to enroll a total of 300 patients aged > or =10 years who fulfil the Ghent criteria for Marfan syndrome. Analyses will be based on intention to treat. CONCLUSION: The results of this clinical trial could lead to profound modification of the management of aortic risk and complications in patients with Marfan syndrome and possibly in patients with thoracic aortic aneurysms of other aetiologies. 2010 Elsevier Masson SAS. All rights reserved.
RCT Entities:
BACKGROUND: Recent studies have demonstrated that blockade of the angiotensin II type 1 receptor with losartandecreases aortic damage in an animal model of Marfan syndrome (a KI mouse model with a pathogenic mutation in the gene coding for fibrillin-1). AIMS: To demonstrate a beneficial effect of losartan on aortic dilatation when added to optimal therapy in patients with Marfan syndrome. METHODS: This is a multicentre, randomized, placebo-controlled, double-blind, clinical trial with a 2-year inclusion period and a 3-year follow-up period. Aortic root diameter will be measured using two-dimensional echocardiography. Secondary endpoints will include incidence of aortic dissection, aortic root surgery, death, quality of life, tolerance and compliance with treatments. We aim to enroll a total of 300 patients aged > or =10 years who fulfil the Ghent criteria for Marfan syndrome. Analyses will be based on intention to treat. CONCLUSION: The results of this clinical trial could lead to profound modification of the management of aortic risk and complications in patients with Marfan syndrome and possibly in patients with thoracic aortic aneurysms of other aetiologies. 2010 Elsevier Masson SAS. All rights reserved.
Authors: Ronald V Lacro; Harry C Dietz; Lynn A Sleeper; Anji T Yetman; Timothy J Bradley; Steven D Colan; Gail D Pearson; E Seda Selamet Tierney; Jami C Levine; Andrew M Atz; D Woodrow Benson; Alan C Braverman; Shan Chen; Julie De Backer; Bruce D Gelb; Paul D Grossfeld; Gloria L Klein; Wyman W Lai; Aimee Liou; Bart L Loeys; Larry W Markham; Aaron K Olson; Stephen M Paridon; Victoria L Pemberton; Mary Ella Pierpont; Reed E Pyeritz; Elizabeth Radojewski; Mary J Roman; Angela M Sharkey; Mario P Stylianou; Stephanie Burns Wechsler; Luciana T Young; Lynn Mahony Journal: N Engl J Med Date: 2014-11-18 Impact factor: 91.245
Authors: Jason R Cook; Luca Carta; Ludovic Bénard; Elie R Chemaly; Emily Chiu; Satish K Rao; Thomas G Hampton; Peter Yurchenco; Kevin D Costa; Roger J Hajjar; Francesco Ramirez Journal: J Clin Invest Date: 2014-02-17 Impact factor: 14.808