Type A Aortic Dissection in Marfan Syndrome: A Case for More Aggressive and Extensive Surgery at the Time of Initial Surgical Operation
In the manuscript by Rylski et al.1 in this issue of Circulation, the authors investigate the long-term outcomes of repair for Type A aortic dissection in Marfan's patients.
Marfan's syndrome (MFS) is one of the genetic syndromes associated with thoracic aortic aneurysms and aortic dissection. The syndrome is the results of fibrillin-1 gene mutations (FBN-1) which is an autosomal dominant mutation with variable expression. Fibrillin 1 is a large glycoprotein that serve as a structural component of calcium-binding microfibrils. These microfibrils are present in elastic connective tissue, such as the medial layer of the ascending aorta as well as in non-elastic connective tissue trough ought the entire body.
- Received February 10, 2014.
- Accepted February 18, 2014.