Thoracic Endovascular Aortic Repair for Aortic Dissection and Thoracic Aortic Aneurysms in Patients With Connective Tissue Disorders

Document Type

Conference Proceeding

Publication Date

9-1-2025

Publication Title

J Vasc Surg

Abstract

Objectives Open surgical repair continues to be the standard of care for treating aortic dissection and thoracic aortic aneurysms (TAA) in patients with connective tissue disorders (CTD). Data on the safety and durability of thoracic endovascular aortic repair (TEVAR) in this patient population remain limited. This study aimed to evaluate the intermediate-term outcomes of TEVAR in patients with CTD. Methods Patients with hereditary CTD who underwent TEVAR for aortic dissection and TAA were identified in the Vascular Quality Initiative between 2014 and 2024. CTDs included Marfan syndrome, Loeys-Dietz syndrome, and Ehlers-Danlos syndrome. Cox regression analysis was used to evaluate the primary outcomes of aneurysmal degeneration, aortic reintervention, dissection propagation, and survival. Aneurysmal degeneration was defined as a ≥ 5-mm increase in aortic diameter from baseline, whereas dissection propagation included either antegrade or retrograde dissection. Results A total of 330 patients (209 males, 121 females) with a mean age of 49 years were included. The most prevalent CTD was Marfan syndrome (85%), followed by Loeys-Dietz syndrome (13%) and Ehlers-Danlos syndrome (2%). TEVAR was performed for dissection in 208 patients (63%) and for TAA in 122 patients (37%). The median maximum aortic diameter was 49 mm for dissection and 60 mm for TAA. Spinal cord ischemia and perioperative mortality were observed in 2% of patients. At 1 year, among patients treated for aortic dissection, aneurysmal degeneration was observed in 28% [18%-37%], dissection propagation in 8% [2%-13%], and aortic reintervention in 28% [20%-35%] (Fig 1). For patients treated for TAA, aneurysmal degeneration occurred in 20% [9%-30%] (Fig 2), dissection in 12% [0%-23%], and aortic reintervention in 27% [16%-37%]. Survival rates at 1 and 2 years were 88% and 86%, respectively, for dissection, and 92% and 87%, respectively, for TAA. Conclusions TEVAR can be safely performed in CTD patients but carries a significant risk of intermediate-term aortic complications. Reinterventions are frequent particularly within the first postoperative year. These findings suggest that TEVAR use in CTD patients should be restricted to emergency cases until improved and durable outcomes can be achieved.

Volume

82

Issue

3

First Page

2

Find It @ Sladen

Share

COinS