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

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%; pConclusions 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.