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)

LOSARTAN

Antihypertensive drug, on the market since 1994

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

Control Losartan 0.0 0.5 1.0 1.5 2.0

p = 0.014 (mm/3 years)

Control (n=31) Losartan (n=26) 0.0 0.5 1.0 1.5

p = 0.033

(mm/3 years)

Aortic root Aortic arch

In patients with an

• perated aortic root

In patients with a native aortic root

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