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Ann Thorac Surg 2009;87:1949-1951. doi:10.1016/j.athoracsur.2008.10.049
© 2009 The Society of Thoracic Surgeons
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Case Reports

Total Aortic Replacement in Loeys-Dietz Syndrome

J. Scott Rankin, MDa, Alan C. Braverman, MDb, Nicholas T. Kouchoukos, MDc,*

a Centennial Medical Center and Vanderbilt University, Nashville, Tennessee
b Washington University School of Medicine, St. Louis, Missouri
c Missouri Baptist Medical Center, St. Louis, Missouri

Accepted for publication October 21, 2008.

* Address correspondence to Dr Kouchoukos, 3009 N Ballas Rd, Suite 360C, St. Louis, MO 63131 (Email: ntkouch{at}aol.com).

Loeys-Dietz syndrome presents early in life with rapidly progressive aortic aneurysmal disease, hypertelorism, and bifid uvula/cleft palate. Genetic testing reveals transforming growth factor-β 1 and 2 mutations. Patients require monitoring for progressive aneurysmal disease, and may need total aortic replacement. Two patients are presented who typify these concepts.






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