Abstract
Objective
To assess the tolerability and efficacy of the investigational use of the angiotensin
II receptor blocker losartan added to β-blockade (BB) to prevent progressive aortic
root dilation in patients with Marfan syndrome (MFS).
Patients and Methods
Between May 1, 2007, and September 31, 2011, 28 patients with MFS (11 males [39%];
mean ± SD age, 13.1±6.3 years) with recognized aortic root dilation (z score >2.0) and receiving BB (atenolol or propranolol) treatment were enrolled. They
were randomized to receive BB (BB: 13 patients) or β-blockade and losartan (BB-L:
15 patients) for 35 months.
Results
In the BB-L group, aortic root dilation was reduced with treatment, and the annual
dilation rate of the aortic root was significantly lower than that of the BB group
(0.10 mm/yr vs 0.89 mm/yr; P=.02). The absolute aortic diameters at the sinus of Valsalva, annulus, and sinotubular
junction showed similar trends, with a reduced rate of dilation in the BB-L group
(P=.02, P=.03, and P=.03, respectively). Five patients (33%) treated with BB-L were noted to have a reduced
aortic root diameter. However, the differences between the groups regarding changes
in aortic stiffness and cross-sectional compliance were not statistically significant.
Conclusion
This randomized, open-label, active controlled trial mostly based on a pediatric population
demonstrated for the first time that losartan add-on BB therapy is safe and provides
more effective protection to slow the progression of aortic root dilation than does
BB treatment alone in patients with MFS.
Trial Registration
clinicaltrials.gov Identifier: NCT00651235.
Abbreviations and Acronyms:
ARB (angiotensin II receptor blockade), AT1 (angiotensin II type 1), BB (β-blockade), BB-L (BB and losartan), MFS (Marfan syndrome), TGF-β (transforming growth factor β)To read this article in full you will need to make a payment
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Article Info
Publication History
Published online: January 14, 2013
Footnotes
Grant Support: This work was supported by a grant from the National Clinical Trial and Research Center of National Taiwan University Hospital ( NCTRC 200604 ).
Identification
Copyright
© 2013 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
