Selective endovascular aortic repair in connective tissue disease patients within a multidisciplinary program achieves comparable outcomes in high-risk patients.

IntroductionEndovascular aortic repair (ENDO) in patients with connective tissue disease (CTD) has historically been discouraged in society guidelines because of durability concerns. However, high-volume aortic centers increasingly employ ENDO strategies in carefully selected high-risk patients across scenarios such as rupture, dissection-related pathology, or hostile anatomy. Herein, we describe temporal ENDO adoption at our multidisciplinary CTD aortic program.MethodsIn this study, 92 patients with CTD undergoing aortic intervention at a single high-volume aortic center between 2005 and 2023 were analyzed. Patients were stratified by ENDO (n = 50) vs open repair (OPEN; n = 42). The primary outcome was freedom from aortic-related mortality (ARM). The secondary outcomes included technical success, complications, secondary aortic intervention (SAI), and overall survival. A prespecified contemporary analysis (2018-2023) was conducted to evaluate the outcomes following the establishment of a multidisciplinary CTD aortic program. The Kaplan-Meier method was used to estimate survival and freedom from SAI.ResultsThe 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%). Patients who underwent ENDO 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 these patients were deemed unfit for open repair by the multidisciplinary aortic team because of physiologic risk, anatomic complexity, or urgent presentation. The technical success rate was lower after ENDO (82% vs 98%; P = .02), and the 30-day reintervention rate was higher (16% vs 0%; P = .01). The median follow-up period was 2.3 years [interquartile range, 1.1-4.7]. SAIs 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). In the full cohort, patients undergoing ENDO had lower 3-year survival rates (71% ± 7% vs 92% ± 4%; log-rank P = .03). In the contemporary cohort, survival at 2 years following ENDO (n = 17) was poorer than that after OPEN (n = 42), although the difference did not reach significance (71% ± 14% vs 92% ± 4%; log-rank P = .1). Similarly, the rate of freedom from ARM was lower, but not significantly different (88% ± 12% vs 98% ± 2%; log-rank P = .4).ConclusionsOpen repair remains the gold standard for connective tissue disease-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.