This Drug Could Reduce The Danger of a Potentially Lethal Complication of Marfan Syndrome

According to a story from Healio, a recent study found that irbesartan, marketed under the name Avapro, can help reduce the rate at which the aorta, the largest blood vessel in the human body, dilates in patients with Marfan syndrome. Irbesartan is often used to treat hypertension (high blood pressure).

About Marfan Syndrome

Marfan syndrome is a genetic disorder which affects connective tissue. The severity of symptoms can vary from person to person. This syndrome is caused by mutations affecting the FBN1 gene. People with Marfan syndrome tend to be tall and thin, with distinctively long limbs, hands, feet, and digits. They also often have exceptionally flexible joints, and scoliosis is also common. Abnormalities of the aorta and heart may also be present, and these are the most dangerous characteristics of the syndrome. Marfan syndrome patients are at risk of aortic aneurysm, in which this artery expands to over 1.5 times its normal diameter. If it ruptures, it can be a medical emergency that is often fatal. Management may involve medication and surgery to prevent the possibility of heart problems or aortic aneurysm and rupture. Keeping heart rate and blood pressure low is beneficial, and patients are discouraged from high intensity physical activity. To learn more about Marfan syndrome, click here.

About The Study

In the study, patients were treated with irbesartan and compared to a control group of patients that received placebo. Then these groups were monitored for a period of five years. The study found that the patients that took irbesartan had an average aortic root diameter of 34.8mm, whereas the control’s average was 35.3mm. While this may seem like only a minor difference, it is nevertheless medically significant.

The rate of expansion in diameter per year was .53mm for the irbesartan group and .74mm for the control group. As an antihypertension drug, it would make sense that irbesartan would be an effective and useful treatment for Marfan syndrome patients, and it was able to keep blood pressure down in patients during the study as well, which is another useful benefit.

Still the authors say that while the study suggests that the drug could be beneficial for Marfan syndrome patients, more study is warranted.


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