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ARBs slow aortic-root dilation in Marfan's syndrome
26 June 2008
MedWire News: Patients with Marfan's syndrome can benefit from treatment with angiotensin receptor II blockers (ARBs), study results indicate.
The main cause of premature death in patients with Marfan's syndrome is progressive enlargement of the aortic root leading to dissection.
Recent studies suggest that aortic-root enlargement is caused by excessive signaling by transforming growth factor (TGF)-β, which could be reduced by treatment with TGF-β antagonists, including angiotensin II-receptor blockers (ARBs), say the researchers.
Harry Dietz (Johns Hopkins University School of Medicine, Baltimore, Maryland, USA) and colleagues evaluated the clinical response to ARBs in 18 patients with Marfan's syndrome with an average age of 6.5 years who had severe aortic-root enlargement.
The patients were followed-up for a median of 26 months, and had a median of five echocardiograms during that period.
Treatment with ARBs significantly decreased the mean rate of change in aortic-root diameter, from 3.54 mm per year during previous medical therapy to 0.46 mm per year during ARB therapy (p<0.001).
The deviation of aortic-root enlargement from normal, as defined by the rate of change in z scores, was reduced by a mean difference of 1.47 z scores per year (p<0.001) after the initiation of ARB therapy.
The sinotubular junction, which is prone to dilation in Marfan's syndrome, also showed a reduced rate of change in diameter with ARB therapy (p<0.05). ARB treatment did not affect aortic segments that are not usually dilated in Marfan's syndrome.
Dietz et al conclude in the New England Journal of Medicine: "These findings suggest that ARBs do not arrest aortic growth but specifically reduce the pathologic rate of increase in the diameter of aortic segments that are already of sufficient size to accommodate the physiologic demands of the tissues for blood flow."
They add that "these findings require confirmation in a randomized trial."
