Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease.

@article{Williams2007EarlySE,
  title={Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease.},
  author={Jason A. Williams and Bart Loeys and Lois U Nwakanma and Harry C Dietz and Philip J. Spevak and Nishant D Patel and Katrien François and Julie Debacker and Vincent L. Gott and Luca A. Vricella and Duke E. Cameron},
  journal={The Annals of thoracic surgery},
  year={2007},
  volume={83 2},
  pages={S757-63; discussion S785-90}
}
BACKGROUND Loeys-Dietz syndrome (LDS) is a recently described genetic aortic aneurysm syndrome resulting from mutations in receptors for the cytokine transforming growth factor-beta. Phenotypic features include a bifid uvula, hypertelorism, cleft palate, and generalized arterial tortuosity, but risk of thoracic aortic rupture and dissection is the principle… CONTINUE READING