Endovascular Aortic Therapy in Patients with Connective Tissue Disorders

P0124 

Abstract Submission 

John Iguidbashian (1), Adam Carroll (2), Michael Kirsch (3), T. Brett Reece (4), Muhammad Aftab (5)

(1) University of Colorado, United States, (2) University of Colorado Anschutz, Denver, CO, (3) University of Colorado, Aurora, CO, (4) University of Colorado Hospital, Aurora, CO, (5) University of Colorado, Anschutz Medical Center, Aurora, Colorado, Aurora, CO

John Iguidbashian    -  Contact Me
University of Colorado

Adam Carroll    -  Contact Me
University of Colorado Anschutz

Michael Kirsch    -  Contact Me
University of Colorado

*T. Brett Reece    -  Contact Me
University of Colorado Hospital

*Muhammad Aftab    -  Contact Me
University of Colorado, Anschutz Medical Center, Aurora, Colorado

John Iguidbashian    -  Contact Me
University of Colorado

Objective: Thoracic endovascular aortic repair (TEVAR) in patients with connective tissue disorders remains controversial given the relative paucity of follow-up data and concern for durability. We report our mid-term outcomes in endovascular thoracic aortic repair in patients with connective tissue disorders.

Methods: A single institution, retrospective review of patients with connective tissue disorders who underwent TEVAR between February 2017 and June 2023. Peri-operative outcomes (cerebrovascular accident (CVA), spinal ischemia, acute kidney injury, 30-day readmission, 30-day mortality) and mid-term follow-up data (mortality, presence of endoleak, and re-intervention rate) were collected and analyzed.

Results: Six patients with connective tissue disorders underwent TEVAR at our institution during the study period. Median age was 39 [IQR: 32.5-58.25] and the majority were male (4/6, 66.7%). Four patients had Marfan Syndrome (66.7%) and two had Loeys-Dietz Syndrome (33.3%). Pre-operative indications for repair included degenerative aneurysmal disease (1/6, 16.7%) and type B dissection (5/6, 83.3%). Half of the cohort had prior aortic surgery (3/6, 50%). First, we evaluated early post-operative outcomes which identified no peri-operative CVA (0/6, 0%), acute kidney injury (0/6, 0%), evidence of spinal ischemia (0/6, 0%), or 30-day mortalities. The average post-operative length of stay was 3.67 days (0.816). Median follow-up interval was 2.47 years (0.96-5.35). Surveillance imaging revealed no graft endoleaks (0/6, 0%), no patients required re-intervention (0/6, 0%), and there were no mortalities (0/6, 0%).

Conclusions: Endovascular therapy for thoracic aorta pathologies in patients with connective tissue disorders is safe and durable based on our institutional experience. TEVAR can be an effective modality in select cases within this patient population. This case series demonstrates success in the midterm time interval, however continued follow-up is necessary to evaluate the long-term durability of this approach.

Descending/Thoracoabdominal Aorta