National Registry Insights on Genetic Aortopathies and Thoracic Endovascular Aortic Interventions

Objective Thoracic endovascular aortic repair (TEVAR) in patients with genetic aortopathies (GA) is controversial given concerns of durability. We describe characteristics and outcomes following TEVAR in patients with GA. Methods All patients undergoing TEVAR between 2010-2023 in the VQI were identified and categorized as having a GA or not. Demographics, baseline, and procedural characteristics were compared among groups. Multivariable logistic regression was used to evaluate the independent association of GA with postoperative outcomes. Kaplan-Meier methods and multivariable Cox-regression analyses were used to evaluate 5-year survival and 2-year reinterventions. Results Of 19,340 patients, 304 (1.6%) had GA (87% Marfan; 9% Loeys-Dietz; and 4% Vascular Ehlers-Danlos). Compared with non-GA, GA patients were younger (50 [37-72] years vs. 70 [61-77] years), more often presented with acute dissection (28% vs. 18%), post-dissection aneurysm (48% vs. 17%), had symptomatic presentation (50% vs. 39%), and were less likely to have degenerative aneurysms (18% vs. 47%) or PAU [+ IMH] (3% vs. 13%) (all p<.001). GA patients were more likely to have prior repair of the ascending aorta/arch (open: 56% vs. 11%;p<.001; endovascular 5.6% vs. 2.1%;p=.017) or the descending thoracic aorta (open: 12% vs. 2%;p=.007; endovascular 8.2% vs. 3.6%;p=.011). No significant differences were found in prior abdominal suprarenal repairs, however, GA patients had more prior open infrarenal repairs (5.3% vs. 3.2%), but fewer prior endovascular infrarenal repairs (3.3% vs. 5.5%)(all p<.05).After adjusting for demographics, comorbidities, and disease characteristics, patients with GA had similar odds of perioperative mortality (4.6% vs. 7.0%; aOR:1.1 [95%CI: 0.57-1.9];p=.75), any in-hospital complication (26% vs. 23%; aOR:1.24 [0.92-1.6];p=.14), or in-hospital reintervention (13% vs. 8.3%; aOR:1.25 [0.84-1.8];p=.25) compared with non-GA patients. However, GA patients had higher likelihood of post-operative vasopressors (33% vs. 27%; aOR:1.44 [1.1-1.9];p=.006) and transfusion (25% vs. 23%; aOR:1.39 [1.03-1.9]; p=.006). 2-year reintervention rates were higher in GA patients (25% vs. 13%; aHR:1.99 [1.4-2.9];p<.001), but 5-year survival was similar (81% vs. 74%; aHR:1.02 [0.70-1.5];p=.1). Conclusions TEVAR for GA patients appeared to be initially safe with similar odds for in-hospital complications, in-hospital reinterventions, and perioperative mortality, as well as similar hazards for 5-year mortality compared with non-GA patients. However, GA patients had higher 2-year reintervention rates. Future studies should assess long-term durability after TEVAR compared with the recommended open repair to appropriately weigh risks and benefits of endovascular treatment in GA patients.