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.

Clinical Impact
Open surgery remains the standard for aortic pathology in Marfan and Loeys-Dietz syndromes, but this report shows that emergent branched endovascular repair can be lifesaving in ruptured thoracoabdominal aneurysms. For clinicians, it is appropriate to consider multibranched endografts as a rescue or bridging strategy in select patients, especially after prior aortic surgery and in centers with significant experience with complex endograft repair. The case studies also demonstrate that branched endograft repair is technically feasible despite a collapsed true lumen, while preserving visceral perfusion and enabling staged reconstruction. Lifelong imaging surveillance and readiness for reintervention remain essential.