Angiotensin Receptor Blockade in Marfan Syndrome
Lila Worden
David A. Sweetser
Angiotensin II Blockade and Aortic-Root Dilation in Marfan’s Syndrome
Brooke BS, Habashi JP, Judge DP, et al. N Engl J Med. 2008;358(26):2787–2795
Background
Aortic dissection is the leading cause of premature death in patients with Marfan syndrome, a connective tissue disorder caused by a fibrillin gene mutation affecting 1:5,000 people. Beta-blockers (BB) are used to reduce the rate of aortic dilation, delaying need for surgical intervention. Mouse models demonstrated that excess transforming growth factor-β (TGF-β) underlies the pathologic aortic changes. TGF-β blockade with angiotensin II receptor blockers (ARB) had been shown to reduce aortic dilation in mice, but evidence in humans was absent.1
Objectives
To evaluate the effect of ARBs on rate of progression of aortic dilation in patients with Marfan syndrome.
Methods
Retrospective cohort study at a single US center from 1996 to 2007.
Patients
18 patients ages 14 months to 16 years with severe Marfan syndrome who were started on ARB based on intolerance of other agents or clinical need (rapid rate of dilation or severe aortic root enlargement). 65 patients on BB monotherapy with mild aortic dilation served as controls. Select exclusion criteria: medication nonadherence.
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