Thoracic endografting in a patient with hereditary hemorrhagic telangiectasia presenting with a descending thoracic aneurysm.

Journal Article (Journal Article)

A 53-year-old woman with no classic risk factors for aneurysm disease presented with the sudden onset of chest pain and dyspnea. A large descending thoracic aortic aneurysm with focal type B dissection was identified and excluded by emergency thoracic endografting. Further postoperative evaluation revealed a history of epistaxis, perioral telangiectasias, hepatic hypervascularity, and a mutation in the gene expressing activin receptor-like kinase 1 (ALK1), leading to a diagnosis of hereditary hemorrhagic telangiectasia. Aortic aneurysms associated with hereditary hemorrhagic telangiectasia are extremely rare, and to our knowledge, this is the first report of thoracic endografting in this patient population.

Full Text

Duke Authors

Cited Authors

  • Andersen, ND; Dubose, J; Shah, A; Lee, T; Wechsler, SB; Hughes, GC

Published Date

  • February 2010

Published In

Volume / Issue

  • 51 / 2

Start / End Page

  • 468 - 470

PubMed ID

  • 20141966

Electronic International Standard Serial Number (EISSN)

  • 1097-6809

Digital Object Identifier (DOI)

  • 10.1016/j.jvs.2009.08.058


  • eng

Conference Location

  • United States