Total arch replacement using frozen elephant trunk and repair of bilateral subclavian artery aneurysms in a patient with type 2 Loeys-Dietz syndrome

Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder. Vessel tortuosity and aneurysms throughout the vasculature are unique to LDS. Aortic root enlargement is ubiquitous, with most patients undergoing root replacement at some point in their lifetime. Multiple vascular procedures are required to prolong life expectancy. We describe a staged hybrid approach to a 17-year-old patient with LDS presenting with ascending aorta, arch, and bilateral subclavian artery aneurysms and prominent tortuosity. Transposition of the left vertebral and subclavian arteries onto the common carotid artery was performed. Total aortic arch replacement with frozen elephant trunk extension into the descending thoracic aorta was performed as a second stage. Bilateral subclavian artery aneurysms were excluded with the use of a four-branched graft.