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Drug Combo Helps Fight Marfan Syndrome

Inherited condition can lead to sudden cardiac death

TUESDAY, Oct. 2 (HealthDay News) -- Treatment with the heart drug perindopril, along with a beta blocker, may help reduce certain cardiac complications of the hereditary disorder Marfan syndrome, research shows.

Marfan syndrome, which principally affects connective tissues, is often characterized by excessive bone elongation and joint flexibility, and eye and cardiovascular system abnormalities. Progressive dilation and rupture of a major blood vessel, the aorta, are the most serious complications and the most common cause of premature death in people with the disorder, according to background information in the study.

Beta blockers are the current standard treatment for Marfan syndrome, but may not be as effective in treating aortic wall degeneration as other therapies. Previous research has found that ACE inhibitors such as perindopril help reduce arterial stiffness.

Reporting in the Oct. 3 issue of the Journal of the American Medical Association, Australian researchers at the Baker Heart Research Institute examined the effectiveness of perindopril in adult Marfan syndrome patients receiving standard beta-blocker therapy. Ten patients received 8 milligrams/day of perindopril while seven others received a placebo for 24 weeks.

"The major novel finding of our study was that perindopril therapy for 24 weeks reduced aortic diameters relative to placebo in both systole (the contraction of the chambers of the heart) and diastole (the expanding of the chambers of the heart) in patients with Marfan syndrome taking standard beta-blocker therapy," the study authors wrote.

"In systole, perindopril reduced the progression of aortic dilatation observed in the placebo group. However, in diastole, perindoperil actually reduced aortic diameters below baseline levels," they noted.

The researchers recommended a larger, longer-term clinical trial.

More information

The March of Dimes Birth Defects Foundation has more about Marfan syndrome.

-- Robert Preidt

SOURCE: Journal of the American Medical Association, news release, Oct. 2, 2007

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