Emergent Endovascular Repair of Ruptured Thoracoabdominal Aortic Aneurysms in Connective Tissue Disorders

Patients with connective tissue disorders such as Marfan and Loeys-Dietz syndromes face a high risk of aortic dissection and rupture at a young age. Open surgical repair is the standard treatment for aortic aneurysms in these patients due to concerns about tissue fragility and long-term durability of endografts. However, in emergent circumstances or patients with prohibitive surgical risk, thoracic endovascular aortic repair with branched stent grafts may be lifesaving. We describe 2 cases of ruptured thoracoabdominal aortic aneurysms in a Marfan and a Loeys-Dietz patient, managed emergently with a 4-branch endograft (Cook Zenith t-Branch). Clinical data were retrospectively reviewed for presentation, procedural details, and outcomes. Both patients presented with acute aortic rupture and collapsed true lumens. Endovascular repair with an off-the-shelf t-Branch device was successfully performed, preserving blood flow to the visceral arteries via branch stent grafts. In selected emergent cases, branched endografts can be used to stabilize life-threatening aortic ruptures in patients with connective tissue disorders, with acceptable early outcomes. These cases underscore that while open surgery remains first-line for elective management, endovascular solutions can serve as a vital bridge in acute situations. Lifelong surveillance is mandatory given the high reintervention rates in this population.