Losartan in Marfan syndrome

Marfan syndrome (MFS) is a multisystemic disorder of the connective tissue with a prevalence of 1 per 5000 and is caused by mutations in the FBN1 gene. Cardiovascular characteristics of MFS include aortic dilatation, aortic dissection and a diminished ventricular function. Transforming growth factor–β (TGF-β) signaling is upregulated in mice with a FBN1 mutation and treatment of these mice with losartan attenuates TGF-β signaling, prevents aortic media degeneration and aortic dilatation.
This thesis focuses on aortic dilatation, ventricular function and type B aortic dissection in adults with MFS and the effect of losartan on these cardinal features.