in adult patients with Marfan syndrome Marfan: connective tissue - PowerPoint PPT Presentation

Effect of losartan on aortic dilatation rate in adult patients with Marfan syndrome • Marfan: connective tissue disorder • Aortic dilatation => aortic dissection and sudden death • Fibrillin-1 defect – Structural dysfunction of media – Regulatory dysfunction (TGF- β) Antihypertensive drug, on the market since 1994 LOSARTAN

Methods • Enrollment: – January 2008 - December 2010 • All (4) Dutch university Marfan screening centres • National database of adults with congenital heart disease (CONCOR) • Randomisation: – Losartan 100 mg vs. no losartan, – Previously prescribed medication was continued • MRI scan at inclusion and after 3 years of follow-up

Only 1 vascular prothesis allowed (mostly aortic root, 27% of population) Randomisation 116 losartan 117 control 113 105

Aortic dilatation rates in 3 years 2.0 1.5 p = 0.033 p = 0.014 1.5 (mm/3 years) (mm/3 years) 1.0 1.0 0.5 0.5 0.0 0.0 Control (n=31) Losartan (n=26) Control Losartan In patients with an In patients with a operated aortic root native aortic root Aortic root Aortic arch

Conclusions • Addition of losartan to standard of care (betablockers) in adults with Marfan syndrome: Reduces aortic root dilatation rate • After aortic root replacement: Reduces aortic arch dilatation rate European Heart Journal doi:10.1093/eurheartj/eht334