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

• Published in: The Annals of thoracic surgery Vol. 104; no. 1; pp. e39 - e42
• Main Authors: Hashizume, Kenichi, MD, PhD, Shimizu, Hideyuki, MD, PhD, Honda, Masanori, MD, PhD, Inoue, Shinya, MD, PhD, Takaki, Hidenobu, MD, Hayashi, Kanako, MD, Kaneyama, Hiroaki, MD
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Inc 01.07.2017
• Subjects: Adult; Aorta, Thoracic - diagnostic imaging; Aorta, Thoracic - surgery; Aortic Aneurysm, Thoracic - diagnosis; Aortic Aneurysm, Thoracic - etiology; Aortic Aneurysm, Thoracic - surgery; Blood Vessel Prosthesis; Cardiothoracic Surgery; Endovascular Procedures - methods; Female; Humans; Loeys-Dietz Syndrome - complications; Loeys-Dietz Syndrome - diagnosis; Loeys-Dietz Syndrome - surgery; Prosthesis Design; Surgery; Tomography, X-Ray Computed
• ISSN: 0003-4975; 1552-6259
• DOI: 10.1016/j.athoracsur.2017.02.017

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.