In this Article

• What is Loeys-Dietz syndrome?
• What are the signs and symptoms of Loeys-Dietz syndrome?
• Is Loeys-Dietz syndrome inherited?
• What causes Loeys-Dietz syndrome?
• How is Loeys-Dietz syndrome diagnosed?
• How is Loeys-Dietz syndrome treated?
• Loeys-Dietz Syndrome Glossary
• Loeys-Dietz Syndrome Index

How is Loeys-Dietz syndrome treated?

The only treatment for Loeys-Dietz syndrome is surgical repair of the aortic aneurysm. Surgical repair of the aneurysms is generally successful. Since the aneurysms tend to rupture early, early and accurate diagnosis is critical to ensure that affected individuals receive prompt surgical treatment. The genetic test can be of value in identifying which individuals with aortic aneurysms have Loeys-Dietz syndrome, and therefore, should have immediate surgery. In contrast to Loeys-Dietz syndrome, in other inherited syndromes associated with aortic aneurysms, surgery carries a poorer prognosis, and the aneurysms can be managed with medications for a longer period of time before surgery becomes necessary. Studies are ongoing to determine whether drug treatment of the Loeys-Dietz syndrome also may be of value.

References: Loeys BL et al. New England Journal of  Medicine, 2006 Aug 24;355(8):788-98.; National Genetics, 2005 Mar;37(3):275-81.

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