Blood-pressure drug may aid in Marfan syndrome, study says


Bloomberg

A blood pressure medicine may ease weakening in the arteries of patients with Marfan syndrome, a genetic disease that leads to Abraham Lincoln-type lankiness and frail blood vessels, researchers said.

The drug, perindopril, is sold as Coversyl by closely held French drugmaker Les Laboratoires Servier and as Aceon by Solvay SA. It reduced stiffness and widening of the aorta, the body’s main artery, Australian researchers wrote in the Journal of the American Medical Association. The drug is from a class of medications called ACE-inhibitors, which block a chemical that causes blood vessels to contract. Marfan affects at least one in 5,000 people, according to the National Marfan Foundation. Patients have a high risk of developing weakness in blood-vessel walls, called aneurysms, which can fatally rupture. Surgery to repair a fragile aorta, the large artery that carries blood from the heart to the body, is common, but may not prevent bursts. “It is likely that ACE inhibitor therapy would delay the need for surgery and reduce incidence of aortic rupture,” researchers said in the study. “The trial was small, but the dramatic nature of the results provides a valid basis for larger trials.” The 24-week study, by researchers at the Baker Heart Research Institute in Melbourne, Australia, evaluated 17 adults with Marfan who were taking beta-blocker drugs, another type of blood-pressure medication. Perindopril reduced aortic stiffness by up to 60 percent and narrowed the diameter of the aorta by a range of 3 millimeters to 7 millimeters, the study found. Marfan syndrome is a connective tissue disorder marked by unusual height, disproportionately long limbs, loose joints, and abnormalities of the eye and cardiovascular system. Because Lincoln, the 16th U.S. president, shared some of the physical traits associated with Marfan, medical historians theorize that he had the disease. The study was supported by a grant from the National Health and Medical Research Council of Australia. Servier, which is based in Neuilly-sur-Seine Cedex, France, provided perindopril for the study. Solvay is based in Brussels.