Aortic-Related Connective Tissue Disorders

Among the conditions we treat at the Comprehensive Aortic Program at the Frankel Cardiovascular Center are Marfan's syndrome, Ehlers-Danlos syndrome and Loeys-Dietz syndrome, which are connective tissue disorders that can affect the aorta. Connective tissue is found throughout the body and holds the body together, supporting muscles, ligaments and skin, while also helping to control the way the body grows.

Marfan Syndrome

Cardiovascular problems are common in people with Marfan syndrome, a disorder that can occur in many different parts of the body. Nine out of ten people with Marfan syndrome experience cardiovascular problems with the most serious complication being enlargement of the aorta. Aortic dilation and aortic aneurysms are common problems that can result in aortic dissection (tear) or rupture with the most common area affected being the aortic root (aortic segment closest to the heart).

Ehlers-Danlos Syndrome

People with Ehlers-Danlos Syndrome (EDS) have a defect in their connective tissue, leaving them with fragile skin and unstable joints. This is a result of faulty collagen, which acts as a "glue" in the body, adding strength and elasticity to connective tissue. Vascular type EDS can cause aneurysms, arterial dissection, and pseudoaneurysms (“false” aneurysms) and while aneurysm formation is relatively rare in EDS, an aneurysm rupture is unpredictable. Aortic dissections may cause pain and compromise blood flow to the extremities or internal organs. Pseudoaneurysms are a contained rupture of a blood vessel.

Loeys-Dietz Syndrome

Among the four main characteristics commonly seen in people with Loeys-Dietz syndrome are aneurysms visible by imaging techniques. They are most often seen in the aortic root (base of the artery leading from the heart), but can be seen in other arteries throughout the body.

Research Brings New Treatment Options for Aortic Disease

We continue to forge the way in finding new treatment options for aortic diseases. The University of Michigan is home to the International Registry of Aortic Dissection, and several research laboratories that are exploring the molecular and genetic etiologies of aortic diseases. Through patient participation and research, we are helping to advance the best treatment options for all types of aortic diseases.

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