Selective Endovascular Aortic Repair in Connective Tissue Disease Patients Within a Multidisciplinary Program Achieves Comparable Outcomes in High-Risk Patients.

Abstract

Introduction: Endovascular aortic repair (ENDO) in connective tissue disease (CTD) patients has historically been discouraged in societal guidelines because of durability concerns. However, high-volume aortic centers increasingly employ ENDO strategies in carefully selected high-risk scenarios such as rupture, dissection-related pathology, or hostile anatomy. Herein, we describe temporal ENDO adoption at our multidisciplinary CTD aortic program.

Methods: Ninety-two CTD patients undergoing aortic intervention at a single high-volume aortic center between 2005 and 2023 were analyzed. Patients were stratified by ENDO (n=50) versus open repair (OPEN; n=42). The primary outcome was freedom from aortic-related mortality (ARM). Secondary outcomes included technical success, complications, secondary aortic intervention (SAI), and overall survival. A prespecified contemporary analysis (2018-2023) evaluated outcomes following establishment of a multidisciplinary CTD aortic program. Kaplan-Meier methods estimated survival and freedom from SAI.

Results: Mean age was 48±16 years and 34% (n=31) were female. Diagnoses included Marfan syndrome (69%), Loeys-Dietz syndrome (15%), familial aortopathy (11%), and vascular Ehlers-Danlos syndrome (5%). ENDO patients more frequently had prior sternotomy/open cardiac surgery (68% vs. 19%; p<.0001), prior aortic surgery (70% vs. 33%; p=.0007), and urgent presentation (50% vs. 14%; p=.0004). Overall, 60% of ENDO patients were deemed unfit for open repair by the multidisciplinary aortic team because of physiologic risk, anatomic complexity, or urgent presentation. Technical success was lower after ENDO (82% vs. 98%; p=.02), and 30-day reintervention was higher (16% vs. 0%; p=.01). Median follow-up was 2.3 years [IQR 1.1-4.7]. SAI occurred more frequently following ENDO (56% vs. 12%; p=.0001), including planned staged procedures (ENDO 28.6% vs. OPEN 20%; p=1) and unplanned reinterventions (ENDO 71.4% vs. OPEN 80%; p=1) among those requiring SAI. In the full cohort, ENDO patients had lower 3-year survival (71±7% vs. 92±4%; log-rank p=.03). In the contemporary cohort, survival at 2 years following ENDO (n=17) was poorer than after OPEN repair (n=42), although the difference did not reach significance (71±14% vs. 92±4%; log-rank p=.1). Similarly, freedom from ARM was lower but not significantly different (88±12% vs. 98±2%; log-rank p=.4).

Conclusions: Open repair remains the gold standard for CTD-associated aortic disease. In carefully selected high-risk patients, ENDO serves as a complementary strategy with acceptable contemporary survival and comparable freedom from ARM. Higher secondary intervention rates reflect both planned staged repair and unplanned surveillance-detected failure modes, underscoring the need for lifelong follow-up within multidisciplinary programs.