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Angiotensin-Receptor Blockers and Marfan Syndrome

Results of a small, preliminary study of the effects of ARBs on abnormal aortic root enlargement are quite promising.

Marfan syndrome is often accompanied by enlargement of the aortic root, increasing the risk for life-threatening dissection. Findings from animal studies have suggested that the angiotensin II-receptor blocker (ARB) losartan can inhibit molecular events that contribute to the phenotypic features of Marfan syndrome. In the present study, investigators compared the rates of aortic root diameter change in 18 children with Marfan syndrome and severe aortic-root enlargement before and after the initiation of ARB therapy. A control group consisted of 65 children with Marfan syndrome who were not treated with an ARB.

The median age of the patients was 6.5 years, and half of them were boys. The mean aortic root diameter before ARB therapy was 3.7 cm, and the median duration of treatment was 49 months. No changes in heart rate or blood pressure occurred after initiation of the ARB. The mean rate of aortic root enlargement before treatment was 3.54 mm per year; after therapy was started, the mean rate was 0.46 mm per year (P<0.001). The more distal sections of the aorta, usually unaffected by Marfan syndrome, continued to show an age-appropriate growth rate. In the control patients, who received beta-blockers but not ARBs, the mean rate of change was 1.71 mm per year.

Comment: This study provides some preliminary evidence of a benefit from ARBs in slowing aortic root dilation in patients with Marfan syndrome. The findings are strengthened by supporting evidence from animal studies. The authors emphasize the need to confirm these findings in a randomized trial, such as the one sponsored by the National Heart, Lung, and Blood Institute that is currently under way. Appropriate patients should be encouraged to enroll.

— Harlan M. Krumholz, MD, SM

Published in Journal Watch Cardiology June 25, 2008

Citation(s):

Brooke BS et al. Angiotensin II blockade and aortic-root dilation in Marfan’s syndrome. N Engl J Med 2008 Jun 26; 358:2787.

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