Endovascular repair of a dissecting pararenal abdominal aortic aneurysm in a patient with type III Loeys-Dietz syndrome

Abstract

Loeys-Dietz syndrome (LDS) type 3 results from a SMAD3 mutation and is a phenotypically milder variant of LDS with frequent aortic, visceral, and cerebral vascular pathologies and osteoarthritis. Historically, endovascular treatment (endovascular aortic repair [EVAR]) of LDS-related aortic aneurysmal disease with traditional modular bifurcated devices has been limited owing to concerns regarding continued aortic dilation at proximal fixation sites. Furthermore, associated dissection pathology has also precluded traditional modular bifurcated EVAR owing to inadequate proximal infrarenal necks and narrow distal aortic domains leading to compromised contralateral gate opening and cannulation as well as limb flow compromise. To address these barriers to EVAR, we present our approach for the endovascular treatment of a dissecting pararenal abdominal aortic aneurysm using an anatomically fixated, bifurcated, unibody aortic stent graft in a patient with LDS-3.

Keywords

• EVAR
• Loeys-Dietz
• Infrarenal aortic dissection
• smad3
• Mutation