Marfan Syndrome

From the desk of Dr. Grayson Wheatley

"Aortic Tissue Defective By Birth"

Nashville, Tennessee - The aorta - being the major blood vessel of the body - is a living, breathing organ (so to speak) of our bodies.  It has to continuously adapt with every heartbeat.  I am sure that you have felt your own pulse before.  The pulse that you feel is a transmission of blood pressure generated by the ejection of blood from the left side of the heart to the body.

 

Most people's aortas can safely endure the repeated stress and strain of expanding and contracting.  The aorta is composed of many different types of tissues including collagen and elastic tissues.

Some people are born with a specific disorder of a particular type of elastic tissue.  The "fibrillin" gene encodes a specific protein which is integrated into the aortic wall along with other tissues in the body, such as joints and the lens in the eye.  The defective protein compromises the elastic function of the aorta.  These are some of the many hallmark features of Marfan Syndrome patients.

As a result, the aorta is predisposed to degenerate prematurely in Mafran Syndrome patients and can lead to aortic dissections or aortic rupture.

Request a consultation with Dr. Wheatley: 1-615-342-6900

most insurance plans accepted

There is no cure for patients with Marfan Syndrome.  From an aortic perspective, they frequently need multiple open aortic surgeries to repair the sections that degenerate and become aneurysmal.

Some patients require a total resection and replacement of their entire aorta - in a staged approach - to completely eliminate all of the abnormal aortic tissue and eliminate any chance of dying from an aortic rupture or dissection.

There is a specialized blood test that can help identify patients with the genetic defect associated with Marfan Syndrome.  However, there are other connective tissue disorders that can affect other proteins in the aortic wall and lead to similar aortic problems.  More research is needed in the area of connective tissue disorders affecting the aorta because some patients present with early degeneration of the aorta, yet don't have a currently identified genetic defect.

In summary, Marfan Syndrome is a connective tissue disorder as part of spectrum of other genetic defects.

Dr. Grayson Wheatley is a Heart and Vascular Surgeon and Director of Aortic and Endovascular Surgery at TriStar Centennial Medical Center in Nashville, TN

Send Dr. Wheatley a message and he will follow-up with you directly.

The Aortic Center at Centennial Medical Center is pre-eminently qualified and skilled at treating patients with all forms of open aortic surgery and procedures (chest and abdomen) and is prepared to care for all patients with aortic aneurysms, aortic dissections and other types of aortic diseases including aortic transections, aortic pseudoaneurysms and penetrating aortic ulcers.

Contact us today to request your consultation at 1-615-342-6900 or email us info@badaorta.com and we will evaluate your unique aortic problem and provide a customized approach to your specific aortic problem.