Urgent Relining of a Fenestrated Stent-Graft for Symptomatic Type V TAAA due to Late Type IIIB and IIIC Endoleaks in a Patient With Marfan Syndrome Case Report and Literature Review.

Abstract

Objective: Although open repair remains the standard of care for managing connective tissue disorders (CTDs), endovascular repair has emerged as an alternative, especially in patients with prior aortic surgery, using a "graft-to-graft" approach. Notwithstanding, long-term interaction between bridging stent-grafts and arterial wall of target vessels (TVs) remains under-investigated. We present a rare case of late-onset type IIIB/IIIC endoleaks in a symptomatic type V thoracoabdominal aortic aneurysm (TAAA) following fenestrated endovascular aneurysm repair (FEVAR) in a patient with Marfan syndrome (MFS), and a literature review.

Methods: A 55-year-old woman with MFS and a history of multiple cardiovascular surgeries, including previous FEVAR using a graft-to-graft approach, presented with chest pain secondary to a large type V TAAA. Computed tomography angiography revealed type IIIB/IIIC endoleaks due to stent disconnection, stent fracture, and fabric tears involving renal and visceral arteries' bridging stents. An urgent endovascular relining was performed with balloon-expandable stent-grafts, achieving enhanced stent protrusion and increased oversizing. The postoperative course was uneventful, with complete resolution of symptoms and no evidence of spinal cord ischemia. Follow-up imaging confirmed complete aneurysm exclusion and maintained patency of TVs. Additionally, a comprehensive literature review was conducted in accordance with PRISMA guidelines, using the search term "MFS," "type IIIB/IIIC endoleak," and "Fenestrated stent-graft" to investigate the rate of incident type III endoleak. Out of 43 identified reports, 8 studies met the inclusion criteria.

Results: Although infrequent (4%-5.9%), type IIIB/IIIC endoleaks after FEVAR in MFS patients may be underreported. These endoleaks may result from under-investigated interactions between bridging stents and TVs, especially due to the inherent fragility of the aortic wall in CTDs. In redo cases, adjunctive strategies, such as increased stent protrusion and greater oversizing, may be necessary to prevent further disconnections.

Conclusions: Endovascular therapy provides a viable option for managing MFS patients, particularly in challenging redo cases with prohibitive surgical risks. Lifelong aortic surveillance in specialized centers and extended and careful follow-up is essential to assess the risks and the true incidence of high-flow endoleaks, as well as the overall safety and durability of these interventions, especially given the progressive nature of CTDs.Clinical ImpactThis study demonstrated the feasibility, safety, and time- and cost-effectiveness of single-disc vascular plug embolization for directional branches during urgent or emergent branched endovascular aortic aneurysm repair using off-the-shelf devices, even without elongation with balloon-expandable stent-grafts. This approach is particularly relevant in urgent scenarios, where minimizing procedural time is critical for optimizing patient outcomes, as well as in resource-limited settings, where efficient allocation of healthcare resources is essential.