Journal of vascular surgery · 2026
Selective Endovascular Aortic Repair in Connective Tissue Disease Patients Within a Multidisciplinary Program Achieves Comparable Outcomes in High-Risk Patients.
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Ueland Walker R, Mazirka Pavel, Fassler Michael J, Neal Dan, Shahid Zain, Cooper Michol A, Upchurch Gilbert R, Spratt John, Jeng Eric, Beaver Thomas, Martin Tomas, Scali Salvatore T, Back Martin R
Аннотация
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.
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.