A.W. den Hartog
- Losartan in Marfan syndrome
V. de Waard
- Award date
- 9 May 2017
- Number of pages
- Document type
- PhD thesis
- Faculty of Medicine (AMC-UvA)
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.
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