[6] Loeys–Dietz syndrome was identified and characterized by pediatric geneticists Bart Loeys and Harry "Hal" Dietz at Johns Hopkins University in 2005.

There is considerable variability in the phenotype of Loeys–Dietz syndrome, from mild features to severe systemic abnormalities.

[7] There is overlap in the manifestations of Loeys–Dietz and Marfan syndromes, including increased risk of ascending aortic aneurysm and aortic dissection, abnormally long limbs and fingers, and dural ectasia (a gradual stretching and weakening of the dura mater that can cause abdominal and leg pain).

Presence of split uvula is a differentiating characteristic from Marfan Syndrome, as well as the severity of the heart defects.

Previous research in laboratory mice has suggested that the angiotensin II receptor antagonist losartan, which appears to block TGF-beta activity, can slow or halt the formation of aortic aneurysms in Marfan syndrome.

A large clinical trial sponsored by the National Institutes of Health is currently underway to explore the use of losartan to prevent aneurysms in Marfan syndrome patients.

Likewise, strenuous physical activity is discouraged in patients, especially weight lifting and contact sports.