Stepwise Total Aortic Repairs With Fenestrated Endografts in a Patient With Loeys-Dietz Syndrome

Ann Thorac Surg. 2017 Jul;104(1):e39-e42. doi: 10.1016/j.athoracsur.2017.02.017.

Abstract

Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) caused by mutations in the gene encoding transforming growth factor-β receptors Ⅰ and Ⅱ. Patients with LDS manifest spontaneous aneurysms and dissections of the aorta and peripheral artery. We report a successful treatment with a hybrid endovascular repair for a rapidly expanding thoracoabdominal aneurysm in a 41-year-old woman affected by LDS. To overcome the difficulties of anatomical and surgical repair, we applied an original strategy using surgeon-modified fenestrated endografts.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aorta, Thoracic / diagnostic imaging
  • Aorta, Thoracic / surgery*
  • Aortic Aneurysm, Thoracic / diagnosis
  • Aortic Aneurysm, Thoracic / etiology
  • Aortic Aneurysm, Thoracic / surgery*
  • Blood Vessel Prosthesis*
  • Endovascular Procedures / methods*
  • Female
  • Humans
  • Loeys-Dietz Syndrome / complications*
  • Loeys-Dietz Syndrome / diagnosis
  • Loeys-Dietz Syndrome / surgery
  • Prosthesis Design
  • Tomography, X-Ray Computed