National registry insights on genetic aortopathies and thoracic endovascular aortic interventions

• Published in: Journal of vascular surgery Vol. 80; no. 4; pp. 1015 - 1024.e7
• Main Authors: Gomez-Mayorga, Jorge L., Yadavalli, Sai Divya, Allievi, Sara, Wang, Sophie X., Rastogi, Vinamr, Straus, Sabrina, Mandigers, Tim J., Black, James H., Zettervall, Sara L., Schermerhorn, Marc L.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.10.2024
• Subjects: Adult; Aged; Aorta, Thoracic - diagnostic imaging; Aorta, Thoracic - surgery; Aortic Aneurysm, Thoracic - diagnostic imaging; Aortic Aneurysm, Thoracic - mortality; Aortic Aneurysm, Thoracic - surgery; Aortic Diseases - diagnostic imaging; Aortic Diseases - mortality; Aortic Diseases - surgery; Blood Vessel Prosthesis Implantation - adverse effects; Blood Vessel Prosthesis Implantation - mortality; Connective tissue disorders; Ehlers-Danlos Syndrome - complications; Ehlers-Danlos Syndrome - diagnosis; Ehlers-Danlos Syndrome - mortality; Endovascular Procedures - adverse effects; Endovascular Procedures - mortality; Endovascular repair; Female; Genetic aortopathy; Genetic Predisposition to Disease; Heritable thoracic aortic disease; Humans; Loeys-Dietz; Loeys-Dietz Syndrome - complications; Loeys-Dietz Syndrome - genetics; Loeys-Dietz Syndrome - mortality; Loeys-Dietz Syndrome - surgery; Male; Marfan syndrome; Marfan Syndrome - complications; Marfan Syndrome - mortality; Middle Aged; Postoperative Complications - etiology; Postoperative Complications - mortality; Registries; Retrospective Studies; Risk Assessment; Risk Factors; Thoracic aortic pathology; Time Factors; Treatment Outcome; United States - epidemiology; Vascular Ehlers-Danlos; United States; Connective tissue disorders; Vascular Ehlers-Danlos; Heritable thoracic aortic disease; Loeys-Dietz; Genetic aortopathy; Thoracic aortic pathology; Marfan syndrome; Endovascular repair
• ISSN: 0741-5214; 1097-6809; 1097-6809
• DOI: 10.1016/j.jvs.2024.05.002

Thoracic endovascular aortic repair (TEVAR) in patients with genetic aortopathies (GA) is controversial, given concerns of durability. We describe characteristics and outcomes after TEVAR in patients with GA. All patients undergoing TEVAR between 2010 and 2023 in the Vascular Quality Iniatitive 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. Of 19,340 patients, 304 (1.6%) had GA (87% Marfan syndrome, 9% Loeys-Dietz syndrome, and 4% vascular Ehlers-Danlos syndrome). Compared with patients without GA, patients with GA were younger (50 years [interquartile range, 37-72 years] vs 70 years [interquartile range, 61-77 years]), more often presented with acute dissection (28% vs 18%), postdissection aneurysm (48% vs 17%), had a symptomatic presentation (50% vs 39%), and were less likely to have degenerative aneurysms (18% vs 47%) or penetrating aortic ulcer (and intramural hematoma) (3% vs 13%) (all P < .001). Patients with GA 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, patients with GA 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%; adjusted odds ratio [aOR], 1.1; 95% confidence interval [CI], 0.57-1.9; P = .75), any in-hospital complication (26% vs 23%; aOR, 1.24; 95% CI, 0.92-1.6; P = .14), or in-hospital reintervention (13% vs 8.3%; aOR, 1.25; 95% CI, 0.84-1.80; P = .25) compared with patients without GA. However, patients with GA had a higher likelihood of postoperative vasopressors (33% vs 27%; aOR, 1.44; 95% CI, 1.1-1.9; P = .006) and transfusion (25% vs 23%; aOR, 1.39; 95% CI, 1.03-1.9; P = .006). The 2-year reintervention rates were higher in patients with GA (25% vs 13%; adjusted hazard ratio, 1.99; 95% CI, 1.4-2.9; P < .001), but 5-year survival was similar (81% vs 74%; adjusted hazard ratio, 1.02; 95% CI, 0.70-1.50; P = .1). TEVAR for patients with GA seemed to be safe initially, with similar odds for in-hospital complications, in-hospital reinterventions, and perioperative mortality, as well as similar hazards for 5-year mortality compared with patients without GA. However, patients with GA had higher 2-year reintervention rates. Future studies should assess long-term durability after TEVAR compared with the recommended open repair to appropriately weigh the risks and benefits of endovascular treatment in patients with GA.