101. Total Aortic Replacement in Loeys-Dietz Syndrome
- Author
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J. Scott Rankin, Alan C. Braverman, and Nicholas T. Kouchoukos
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Adolescent ,Heart Valve Diseases ,Loeys–Dietz syndrome ,Young Adult ,medicine.artery ,Aneurysmal disease ,Internal medicine ,Bifid uvula ,medicine ,Humans ,Hypertelorism ,Genetic testing ,Aorta ,medicine.diagnostic_test ,business.industry ,Syndrome ,medicine.disease ,Aortic Aneurysm ,Surgery ,Cleft Palate ,Uvula ,Aortic Valve ,Circulatory system ,cardiovascular system ,Cardiology ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business - Abstract
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-beta 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.
- Published
- 2009
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