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Outcomes after Thoracic Endovascular Aortic Repair in Patients with Connective Tissue Disorders
Jorge L. Gomez-Mayorga, MD1, Sai D. Yadavalli, MD1, Sara Allievi, MD1, Sophie X. Wang, MD1, Vinamr Rastogi, MD1, Sabrina Strauss, MS2, James H. Black, III, MD3, Sara L. Zettervall, MD MPH4, Marc L. Schermerhorn, MD1.
1Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA, 2UC San Diego (UCSD), San Diego, CA, USA, 3Johns Hopkins University School of Medicine, Baltimore, MD, USA, 4University of Washington, Seattle, WA, USA.

Objective: Endovascular repair in patients with connective tissue disorders (CTD) is controversial given concerns of durability. We describe characteristics and outcomes following TEVAR in patients with CTDs.
Methods: All patients undergoing TEVAR from 2010-2023 in the VQI were categorized as having a CTD or not. Demographics, baseline, and procedural characteristics were compared among groups. Multivariable logistic regression was used to evaluate the independent association of CTD with postoperative outcomes. Kaplan-Meier methods and multivariable Cox-regression analyses were used to evaluate 5-year survival and 3-year reinterventions.
Results: Of 17,422 patients, 261 had CTD (87% Marfan; 8% Loeys-Dietz; and 5% Ehlers-Danlos). Compared with no-CTD, CTD patients were younger (50y vs. 70y), more likely had acute dissection (30% vs. 18%), post-dissection aneurysm (44% vs 17%), symptomatic presentation (53% vs. 39%), and less likely degenerative aneurysms (19% vs. 47%) or PAU [+ IMH] (3% vs. 13%) (all p<.001). CTD patients were more likely to have prior repair of the ascending aorta/arch (open: 51% vs. 10%; endovascular 6% vs. 2%) or the descending aorta (open: 11% vs. 2%; endovascular 9% vs. 3%). No significant differences were found in abdominal suprarenal repairs, however, CTD patients had higher prior open infrarenal repair (5% vs. 3%), but lower prior endovascular infrarenal repair (4% vs. 5%)(all p<0.05). CTD was not significantly associated with perioperative mortality (3.8% vs. 7.1%; aOR:0.88 [95%CI 0.39-1.71];p=.73), any in-hospital complication (aOR:1.18 [0.85-1.60];p=.31), or in-hospital reintervention (aOR:1.29 [0.84-1.91];p=.22) compared with no-CTD. However, CTD patients had higher likelihood of transfusion (≥2PRBC: 33% vs. 30%; aOR:1.42 [1.05-
1.90];p=.028) and vasopressors (33% vs. 27%; aOR:1.44 [1.08-1.91];p=.011). 3-year reintervention rates were higher in CTD patients (31% vs. 17%; aHR:2.08 [1.38-3.15];p<.001), but 5-year survival was similar (86% vs. 79%; aHR:1.18 [0.74-1.87];p=0.47).
Conclusions: TEVAR for CTD 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 no-CTD patients. However, CTD patients had higher 3-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 CTD patients.

Prior Surgical History
Prior Aortic Surgery
Prior open surgery location
Descending thoracic1.9%11.5%.013
Prior endovascular surgery location
Descending thoracic3.4%9.2%.008
Perioperative outcomes
(N=17161)(N=261)P-valueaOR*95% CIP-value
Perioperative death7.1%3.8%0.0530.880.39-1.710.73
Spinal cord ischemia3.3%2.7%0.590.790.31-1.670.58
Postoperative dialysis2.7%2.3%0.720.930.32-2.100.89
Myocardial infarction2.1%0.4%0.090.310.02-1.410.24
Pulmonary complications8.1%6.5%0.410.990.56-1.630.98
Postoperative transfusion23%26%
Any complication†23%26%0.911.10.85-1.600.31
Postoperative spinal drain3.6%6.1%
Postoperative vasopressors27%33%0.0391.41.08-1.900.011
In-hospital reinterventions8.3%14%0.0021.30.82-1.900.22
ICU length of stay, days2 [1-4]3 [2-5]0.10---
Total length of stay, days4 [2-8]6 [4-11]0.051---
Follow up
(N=11756)(N=200)P-valueaHR95% CIP-value
5-year survival79%86%
3-year reinterventions17%31%<.0012.081.38-3.15<.001
Data are presented as median [interquartile range] for continuous measures, and frequencies and percentages for categorical measures.† Any complication was defined as the presence of any of the following during index hospitalization: pulmonary complication, cardiac complication, acute kidney injury (AKI), new dialysis, stroke, intestinal ischemia, leg ischemia, or reoperation. * Multivariable models adjusted for baseline pathology, age, gender, aortic diameter, BMI, race, prior diabetes, prior renal dysfunction

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