Marfan syndrome (MFS) is a hereditary disorder principally affecting the connective tissues of the body, often characterized by excessive bone elongation and joint flexibility and abnormalities of the eye and cardiovascular system. Progressive aortic dilation and rupture are the most serious complications and the most common cause of premature death. Beta-blockers are currently the standard treatment for MFS, but may not be as effective as other therapies in treating aortic wall degeneration, according to background information in the article. Angiotensin-converting enzyme (ACE) inhibitors have been shown to reduce arterial stiffness.
Anna A. Ahimastos, Ph.D., of the Baker Heart Research Institute, Melbourne, Australia, and colleagues conducted a study to examine the effectiveness of the ACE inhibitor perindopril to reduce arterial stiffness and aortic dilation relative to placebo in 17 adult patients with MFS taking standard beta-blocker therapy. The randomized trial began in January 2004 and was completed in September 2006. Patients were administered 8 mg/day of perindopril (n = 10) or placebo (n = 7) 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 MFS taking standard beta-blocker therapy. In systole, perindopril reduced the progression of aortic dilatation observed in the placebo group. However, in diastole, perindopril actually reduced aortic diameters below baseline levels by an average of between 1.2 and 3.0 mm/mē," the authors write.
"In conclusion, therapy with perindopril reduced both aortic stiffness and aortic root diameter in patients with MFS taking standard beta-blocker therapy. These findings warrant further investigation in a larger, longer-term clinical trial."