Which condition is characterized by an increased risk of aortic rupture and dilation?

Marfan Syndrome is characterized by several features that significantly impact the cardiovascular system, particularly the aorta. This connective tissue disorder leads to structural abnormalities in various tissues, including those forming the aorta. Individuals with Marfan Syndrome often exhibit aortic dilation and a predisposition to aortic dissection and rupture due to the weakness in the vessel wall.

The increased risk of aortic rupture and dilation in Marfan Syndrome can be attributed to its effects on fibrillin-1, a crucial protein involved in the elasticity and structural integrity of connective tissue. As a result, the aorta can become enlarged, thin, and more susceptible to tears under pressure, leading to serious complications if not monitored and managed appropriately.

While conditions like Ehlers Danlos Syndrome, Bicuspid Aortic Valve, and Loeys Dietz Syndrome also involve vascular issues and may lead to aortic complications, Marfan Syndrome is most specifically associated with the classic presentation of aortic dilation and the highest risk of rupture due to its well-documented effects on the aorta.