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Contemporary Outcomes of Aortic Surgery in Connective Disease Patients and the Evolving Role for Endovascular Repair in Select High-Risk Patients
Walker R Ueland, Pavel Mazirka, Salvatore T Scali, Zain Shahid, Michol A Cooper, Gilbert R Upchurch, Jr., John R Spratt, Thomas M Beaver, Tomas D Martin, Martin R Back
University of Florida, Gainesville, FL
Introduction: Endovascular aortic repair(ENDO) in connective tissue disease(CTD) patients has been discouraged in societal guidelines due to durability concerns. However, high-volume aortic centers increasingly use ENDO in selected high-risk scenarios such as rupture, dissection, or hostile anatomy. Herein, we describe temporal ENDO adoption at our center, which employs a multidisciplinary CTD patient-care model.
Methods: We retrospectively reviewed 92 CTD patients undergoing aortic intervention from 2005-2023. Patients were stratified by ENDO(n=50) versus Open repair(n=42). Primary outcome was freedom from aortic-related mortality(ARM); secondary outcomes included technical success, complications, secondary aortic intervention(SAI), and survival. Because the ENDO experience was captured from 2005-2023, and a contemporary multidisciplinary CTD aortic program was formally established in December 2017, we report both the full cohort analysis and a pre-specified contemporary subgroup comparison(2018-2023). Multivariable logistic and Cox regression was used for risk adjustment. Kaplan-Meier analysis estimated survival and freedom from SAI.
Results: Mean age was 48±16 years; 34% were female(n=31). Diagnoses included: Marfan syndrome-67%(n=63), Loeys-Dietz-15%(n=14), familial aortopathy-11%(n=10), and Ehlers-Danlos-5%(n=5). ENDO patients more frequently had prior sternotomy/open cardiac surgery(68% vs. 33%;p=.002), prior aortic surgery(70% vs. 33%;p=.007), and non-elective presentation(62% vs. 24%;p=.0004). Complicated type B dissection was the dominant indication(ENDO-48% vs. Open-7%;p=.0001). 88% of ENDO patients were unfit for open repair due to physiologic risk(32%) or anatomic complexity(56%)(
Table). Contemporaneously, ENDO comprised 27% of CTD repairs.
Technical success was lower in the ENDO cohort(82% vs. 98%;p=.019) and 30-day reintervention was higher(16% vs. 0%;p<.05). The most common procedures were TEVAR in the ENDO group(34%) and open ascending/transverse arch(76%) or TAAA repair(24%) in the Open group. Median follow-up was 2.2 years[IQR 1.1-3.9]. SAI occurred more frequently after ENDO(56% vs. 12%;p<.0001), with correspondingly lower 1-year freedom from SAI(51±8% vs. 71±4%;log-rank p<.001;
Figure-A).In the full cohort, early survival was similar; however, at 3-years, ENDO patients had higher all-cause mortality(1-year: ENDO, 94±4% vs. Open, 95±4%; 3-year: ENDO, 73±7% vs. Open 92±4%;log-rank p=.007). In the contemporary cohort(2018-2023), survival among ENDO patients(n=15) was comparable to Open repair(n=42)(2-year: ENDO-87±13% vs. Open, 92±4%;log-rank p=.07). Freedom from ARM did not differ between groups(ENDO-91.7±8% vs. Open-97.4±3%;log-rank p=.4;
Figure-B).
Conclusions: Open repair remains the gold standard for CTD patients; however, ENDO can serve as a life-saving bridge or adjunct in carefully selected high-risk patients. Importantly, contemporary ENDO patient survival was comparable to Open repair and superior to historical outcomes, reflecting advances in patient selection, technical refinement, and the maturation of a multidisciplinary CTD aortic program. Durability concerns persist, highlighting the need for preoperative counseling, vigilant surveillance, and rigorous longitudinal follow-up. These findings support ENDO as a complementary, patient-centered treatment option in select cases that expands therapeutic options without compromising survival.
Table. Patient and procedural characteristics significantly associated with procedure type.
| Feature, No. (%) | | Overall(N=92) | | Endovascular(N=50, 54%) | | Open(N=42, 46%) | | p-value |
| Age, years (SD) | | 47.6 (16.3) | | 49.4 (16.1) | | 45.4 (16.4) | | .3 |
| Female sex | | 31 (33.7) | | 14 (28.0) | | 17 (40.5) | | .3 |
| Connective tissue disease type | | | | | | | | |
| Marfan syndrome | | 63 (68.5) | | 40 (80.0) | | 23 (54.8) | | .01 |
| Vascular Ehlers-Danlos | | 5 (5.4) | | 4 (8.0) | | 1 (2.4) | | .4 |
| Loeys-Dietz syndrome | | 14 (15.2) | | 3 (6.0) | | 11 (26.2) | | .009 |
| Preop anti-coagulation | | 31 (33.7) | | 22 (44.0) | | 9 (21.4) | | .03 |
| Prior sternotomy/open-heart surgery | | 48 (52.2) | | 34 (68.0) | | 14 (33.3) | | .002 |
| Prior aortic surgery | | 49 (53.3) | | 35 (70.0) | | 14 (33.3) | | .0007 |
| # prior aortic surgeries | | 1 [0,1] (0,4) | | 1 [0,1] (0,4) | | 0 [0,1] (0,2) | | .0008 |
| Prior arch repair | | 16 (17.4) | | 13 (26.0) | | 3 (7.1) | | .03 |
| Presentation | | | | | | | | |
| ASA class | | 3.7 (0.65) | | 3.5 (0.71) | | 3.9 (0.50) | | .008 |
| Non-elective | | 41 (44.6) | | 31 (62.0) | | 10 (23.8) | | .0003 |
| Crawford Extent 4 (vs. 1-3) | | 17 (18.5) | | 5 (10.0) | | 12 (28.6) | | .03 |
| Acute aortic syndrome | | 15 (16.3) | | 13 (26.0) | | 2 (4.8) | | .009 |
| Any dissection-related pathology | | 66 (71.7) | | 42 (84.0) | | 24 (57.1) | | .006 |
| Acute type A | | 12 (13.0) | | 7 (14.0) | | 5 (12.0) | | 1.0 |
| Acute type B | | 9 (9.8) | | 8 (16.0) | | 1 (2.4) | | .04 |
| Chronic type B | | 18 (19.6) | | 16 (30.8) | | 2 (4.8) | | .001 |
| Intractable pain | | 26 (28.3) | | 21 (42.0) | | 5 (11.9) | | .002 |
| Rapid expansion | | 16 (17.4) | | 16 (32.0) | | 0 (0) | | .0001 |
| Procedure Type | | | | | | | | |
| TEVAR | | 17 (18.5) | | 17 (34.0) | | 0 (0) | | |
| TEVAR + arch repair or TAAA repair | | 11 (12.0) | | 11 (22.0) | | 0 (0) | | |
| Arch debranching | | 4 (4.3) | | 4 (36.4) | | 0 (0) | | |
| TEVAR + carotid/subclavian bypass | | 13 (14.1) | | 13 (26.0) | | 0 (0) | | |
| F/BEVAR or EVAR | | 9 (9.8) | | 9 (18.0) | | 0 (0) | | |
| Open Arch/TAAA repair | | 38 (41.3) | | 0 (0) | | 38 (90.5) | | |
| Open ascending/arch | | 29 (31.5) | | 0 (0) | | 29 (76.3) | | |
| TAAA repair [Extent 1-4] | | 9 (9.8) | | 0 (0) | | 9 (23.7) | | |
| Open Infra/Suprarenal repair | | 4 (4.3) | | 0 (0) | | 4 (9.5) | | |
| Outcomes | | | | | | | | |
| Technical success* | | 82 (89.1) | | 41 (82.0) | | 41 (97.6) | | .02 |
| 30-day mortality | | 2 (2.2) | | 1 (2.0) | | 1 (2.4) | | 1 |
| Any in-hospital complication | | 25 (27.2) | | 13 (26.0) | | 12 (28.6) | | .8 |
| LOS (days±SD) | | 14.4 (12.5) | | 14.0 (14.0) | | 14.9 (10.6) | | .1 |
| Planned Return to OR | | 5 (6.3) | | 3 (8.3) | | 2 (4.8) | | .7 |
| Unplanned Return to OR | | 33 (41.8) | | 25 (69.4) | | 7 (16.7) | | <.0001 |
| Secondary Aortic Intervention (SAI) | | 28 (30.4) | | 24 (48.0) | | 4 (9.5) | | .0001 |
| SAI within 30-days | | 8 (10.4) | | 8 (16.7) | | 0 (0) | | .02 |
| Time to SAI-months, median [IQR] | | 5.7 [.8,14.4] | | 5.6 [.47,12.3] | | 11.5 [4.3,19.1] | | .4 |
| Any death during follow-up | | 23 (25.0) | | 20 (40.0) | | 3 (7.1) | | .0003 |
*
Technical success = absence of unplanned SAI and/or death within 30-days from the index procedure
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