Abstract
Objective:
To describe a single-center experience of “complete aortic repair” consisting of surgical or endovascular total arch replacement/repair (TAR) followed by thoracoabdominal fenestrated-branched endovascular aortic repair (FB-EVAR).
Methods:
We reviewed 480 consecutive patients who underwent FB-EVAR with physician-modified endografts (PMEGs) or manufactured stent-grafts between 2013 and 2022. From those, we selected only patients treated with open or endovascular arch repair and distal FB-EVAR for aneurysms involving the ascending, arch and thoracoabdominal aortic segments (zones 0-9). Manufactured devices were used under an investigational device exemption protocol. Endpoints included early/in-hospital mortality, mid-term survival, freedom from secondary intervention, and target artery instability.
Results:
There were 22 patients, 14 men and 8 women with a median age of 72±7 years. Thirteen postdissection and 9 degenerative aortic aneurysms were repaired with a mean maximum diameter of 67±11 mm. Time from index aortic procedure to aneurysm exclusion was 169 and 270 days in those undergoing 2- and 3-stage repair strategies, respectively. The ascending aorta and aortic arch were treated with 19 surgical and 3 endovascular TAR procedures. Three (16%) surgical arch procedures were performed elsewhere, and perioperative details were unavailable. Mean bypass, cross-clamp, and circulatory arrest times were 295±57, 216±63, and 46±11 minutes, respectively. There were 4 major adverse events (MAEs) in 2 patients: both required postoperative hemodialysis, 1 had postbypass cardiogenic shock necessitating extracorporeal membrane oxygenation, and the other required evacuation of an acute-on-chronic subdural hematoma. Thoracoabdominal aortic aneurysm repair was performed with 17 manufactured endografts and 5 PMEGs. There was no early mortality. Six (27%) patients experienced MAEs. There were 4 (18%) cases of spinal cord injury with 3 (75%) experiencing complete symptom resolution before discharge. Mean follow-up was 30±17 months in which there were 5 patient deaths—0 aortic related. Eight patients required ≥1 secondary intervention, and 6 target arteries demonstrated instability (3 IC, 1 IIIC endoleaks; 2 TA stenoses). Kaplan-Meier 3-year estimates of patient survival, freedom from secondary intervention, and target artery instability were 78±8%, 56±11%, and 68±11%, respectively.
Conclusion:
Complete aortic repair with staged surgical or endovascular TAR and distal FB-EVAR is safe and effective with satisfactory morbidity, mid-term survival, and target artery outcomes.
Clinical Impact
The presented study demonstrates that repair of the entirety of the aorta - via total endovascular or hybrid means- is safe and effective with low rates of spinal cord ischemia. Cardiovascular specialists within comprehensive aortic teams at should feel confident that staged repair of the most complex degenerative and post-dissection thoracoabdominal aortic aneurysms can be safely performed in their patients with complication profile similar to that of less extensive repairs. Meticulous and intentional case planning is imperative for immediate and long-term success.
Introduction
Patients with diffuse aneurysmal aortic disease often require multiple interventions. Whereas open surgical repair has historically been considered the “gold standard” for reconstruction of arch and thoracoabdominal aortic aneurysms (TAAAs), hybrid and fenestrated-branched endovascular aortic repair (FB-EVAR) has become widely used in the management of these complex lesions owing to decreased perioperative morbidity and mortality compared with conventional open surgical repair.1–8
Since Dr. Hans Borst and colleagues’ initial description of the “elephant trunk” procedure in 1983, numerous advancements in open surgical technique and endograft technology have led to specialized centers using hybrid repair and FB-EVAR for arch and thoracoabdominal aortic disease, respectively, as first-line therapies.9–13 Our institution has adopted a similar strategy for patients with diffuse aortic disease: surgical total arch replacement with frozen elephant trunk (TAR+FET) or total endovascular arch repair followed by distal endovascular TAAA repair with FB-EVAR. 14 The aim of this study is to evaluate the early and mid-term outcomes of patients undergoing complete aortic repair using hybrid and total endovascular techniques.
Methods
Study Design
Approval for this study was granted by the Mayo Clinic Institutional Review Board. Patients included in analysis underwent FB-EVAR with both physician-modified endografts (PMEGs) and manufactured devices, the latter of whom were consented for enrollment in a prospective, nonrandomized, physician-sponsored investigational device exemption (IDE) study (G130030 and G130266) and registered in ClinicalTrials.gov (NCT1937949 and NCT2089607). Patients treated with PMEGs were not enrolled in an IDE study.
Patient demographics, clinical features, cardiopulmonary risk factors, operative and clinical outcomes were collected. For patients registered in the IDE studies, clinical outcomes were adjudicated by a clinical event committee, reviewed by the data safety monitoring board, and reported annually to the US Federal Drug Administration. For those undergoing FB-EVAR with PMEGs, data were retrospectively collected and reviewed. Some patients included in this study have been included in previously published data.14,15
Patient Selection
Patients with disease affecting the thoracic and thoracoabdominal aorta are routinely evaluated by a multidisciplinary team comprised of cardiologists, clinical geneticists, cardiovascular surgeons, and vascular surgeons. A shared-decision making process is used to determine the timing and modality of aortic repair—open, hybrid, or endovascular. Younger patients and those with connective tissue disease are often recommended to undergo conventional open surgical repair. In contrast, older patients, those with significant cardiopulmonary comorbidities and those who have undergone previous aortic repair are assessed for hybrid and EVAR.
Operative Technique and Device Design
Surgical and endograft design were left at the discretion of the primary operators (Figure 1). Details regarding patient selection, operative technique, and device design have been previously described by our group.16–23 Anatomical factors of the aorta, branches, and access vessels were determined using computed tomographic angiography (CTA), and device sizing was performed using centerline reconstruction software (3mensio, Pie Medical Imaging, Maastricht, The Netherlands & Aquarius Intuition, TeraRecon Inc, Foster City, CA, USA). Surgical TAR was typically performed with a prefabricated multi-branched Dacron graft. Dimension and type of FET device varied based on operator preference, commercial availability, and patient anatomy.

Illustrative examples of hybrid (A) and endovascular (B) complete aortic repair.
All patients who received patient-specific or off-the-shelf fenestrated and branched endografts under the IDE protocols were manufactured by Cook Medical Inc. (William Cook, Brisbane, Australia). PMEGs were created using the Zenith platform (Cook Medical, Bloomington, IN) under sterile technique using the same design and implantation principles used for manufactured devices.21–23 Both manufactured and PMEGs were designed with 1 to 5 reinforced fenestrations or directional branches for TA incorporation with options for preloaded guidewires, low-profile fabric, and radio-opaque gold markers. Manufactured patient-specific and off-the-shelf endografts were preferentially used when they were available at our institution. PMEGs were used in the absence of IDE protocol devices or in cases of symptomatic or exceedingly large aneurysms not amenable to off-the-shelf repair.
Neuroprotection
With a greater understanding of the neurologic complications associated with extensive aortic repair including spinal cord ischemia (SCI), our practice evolved over the study period. As our experience and knowledge regarding the risk-benefit profile of spinal drain placement advanced, routine spinal drainage was discontinued in lieu of selective prophylactic and as needed therapeutic drainage. 24 Furthermore, perioperative neurological monitoring matured with the use of motor and somatosensory evoked potentials and near-infrared spectroscopy. A protocol aimed at reducing the incidence of SCI and improving the effectiveness of rescue maneuvers was designed and implemented during the study period. 25 All patients were observed postoperatively in a dedicated intensive care unit with close hemodynamic, neuromuscular, and laboratory monitoring. SCI was diagnosed clinically and confirmed with magnetic resonance imaging. Permissive hypertension was maintained for several weeks postoperatively with goal systolic blood pressure of 130 to 150 mm Hg unless contraindicated by other comorbid conditions.
Surveillance
Follow-up consisted of physical examination, laboratory studies, target artery (TA) duplex ultrasound, and CTA prior to discharge and at 3 and 12 months postoperatively with annual surveillance thereafter. Later in the study period, cone-beam computed tomography (CBCT) was used intraoperatively and replaced predischarge CTA in select patients. CBCT with and without contrast detects a higher rate of technical defects compared to angiography and CTA alone and has become a routine completion study for all standard and complex EVAR procedures at our institution. 26 Patients with nondialysis-dependent kidney disease were evaluated with noncontrast axial imaging and duplex ultrasound.
Endpoints and Statistical Analysis
The Society for Vascular Surgery reporting standards for EVAR involving the mesenteric and renal arteries was used, when possible, to determine variables and endpoints. 27 Endpoints included 30-day/in-hospital mortality and major adverse events (MAEs) (myocardial infarction, cardiogenic shock, acute kidney injury, new-onset dialysis, respiratory failure, bowel ischemia requiring resection, SCI, or major stroke), patient survival and freedom from aortic-related mortality (ARM), freedom from secondary interventions, TA patency, and freedom from TA instability. Estimated intraoperative blood loss of 1 L or greater was not considered a major event.
Normally distributed variables were expressed as mean ± standard deviation (SD). Median and interquartile range (IQR) were used to express variables that did not follow a normal distribution. Pearson χ2 or Fisher exact tests were used for categorical variables. Kaplan-Meier estimates were used to report time-dependent outcomes up to 3 years. Analysis was performed using SPSS (IBM, Armonk, NY) and JMP Pro (SAS, Cary, NC).
Results
Study Patients
During the study period, 480 patients underwent FB-EVAR of the aortic arch, thoracoabdominal and/or abdominal segment, of which 22 (5%) underwent complete repair of the aorta spanning from zone 0 to zone 9. There were 14 men and 8 women with a mean age of 72±7 years old. There were 13 (59%) postdissection and 9 (41%) degenerative aneurysms with a mean maximum diameter of 67±11 mm. A breakdown of patient demographics, risk factors, and anatomical characteristics can be found in Table 1. Two patients intended for complete aortic repair at the time of proximal thoracic aortic intervention experienced 30-day mortality—1 surgical TAR+FET and 1 endovascular arch repair.
Demographics, Cardiovascular Risk Factors, and Anatomic Characteristics of 22 Patients Undergoing Complete Aortic Repair.
Abbreviations: ASA = American Society of Anesthesiologists Physical Status Classification System; BMI = body mass index; CKD = Chronic Kidney Disease; CKD = Chronic Kidney Disease; IQR = interquartile range; TAAA = Thoracoabdominal aortic aneurysm; TIA = Transient Ischemic Attack.
Ascending and Aortic Arch Repair
The ascending and aortic arch aneurysms were treated with 19 (86%) surgical TAR and 3 (14%) endovascular arch procedures. Three (16%) surgical TAR procedures were performed elsewhere; therefore, intraoperative and perioperative details are unavailable for comparative analysis. Of the patients undergoing surgical TAR at our institution (n=16), 8 (50%) had previously undergone open thoracic aortic repair in the form of 5 hemiarch and 3 ascending aortic repairs. The majority (13 [69%]) of patients undergoing surgical TAR underwent FET placement with a mean endograft length of 140±5.8 mm with the remaining patients undergoing classic elephant trunk (n=5 [26%]) and hybrid (n=1 [5%]) extension into the proximal descending thoracic aorta. Mean bypass, cross-clamp, and circulatory arrest times were 295±57, 216±63, and 46±11 minutes, respectively.
Two patients in the surgical TAR group experienced 4 MAEs. One patient who presented with acute type A dissection required extracorporeal membrane oxygenation and hemodialysis for cardiogenic shock and acute renal failure, respectively. Another patient experienced acute-on-chronic subdural hematoma requiring surgical evacuation and acute renal failure necessitating renal replacement therapy.
All patients who received endovascular arch repair (3 patients, 14%) underwent previous ascending aortic repair due to acute type A aortic dissection. Technical success was achieved in 100% of patients with a mean operative time of 217±22 minutes. Two patients received arch endografts with 3 branches and 1 underwent implantation of a 2-branch device with left common carotid to subclavian artery bypass. Inadequate innominate artery landing zone necessitated right common carotid to subclavian artery bypass in 2 (66%) patients. One (33%) patient in the endovascular arch repair group experienced cervical wound skin and soft-tissue infection and prolonged intubation but did not require tracheostomy.
Thoracoabdominal Aortic Repair
There were 16 (72%) extent II and 6 (27%) extent I TAAAs. Fifteen (68%) patients underwent staging with TEVAR performed after arch repair but prior to FB-EVAR. Time from initial aortic procedure to complete aneurysm exclusion was 169 days in those undergoing 2-stage repair and 270 days in those undergoing a 3-stage strategy. TAAAs were treated with 17 manufactured—16 (73%) patient-specific and 1 (5%) off-the-shelf t-Branch—devices and 5 (22%) PMEGs. There were 3.9±0.6 TAs incorporated per patient. Upper extremity access was used in 13 (59%) of cases. Technical success was achieved in 95% (21/22) of patients. Failure to cannulate a celiac axis directional branch after distal graft migration occurred in the single patient with technical failure, with the branch therefore intentionally occluded with an Amplatzer Vascular Plug II (St. Jude Medical, Saint Paul, MN, USA) and the celiac axis subsequently ligated without clinical consequence. Procedural details of FB-EVAR can be found in Table 2.
Procedural Details and Operative Metrics of 22 Patients Undergoing Complete Aortic Repair.
Five (23%) patients experienced MAEs with no 30-day mortality. Four (18%) patients had spinal cord injuries, 3 (75%) with complete symptom resolution prior to hospital discharge. A breakdown of MAEs following each repair stage can be found in Table 3.
Major Adverse Events of 22 Patients Undergoing Complete Aortic Repair.
Abbreviation: MAE = major adverse event.
3 of 19 surgical total arch replacement procedures were performed at an outside institution, and therefore, perioperative details are unavailable.
Temporary dialysis
Mid-Term Outcomes
Mean follow-up was 30±17 (35, 9-41) months with 5 (23%) patient deaths—zero aortic related. Three-year Kaplan-Meier patient survival estimate was 77±8% (Figure 2A). Eight (36%) patients required ≥1 secondary intervention and 6 (7%) TAs demonstrated instability. All instances of TA instability occurred in the thoracoabdominal segment with no arch TA endoleak, stenosis, kink, or occlusion. There were 3 type IC and 1 type IIIC endoleak all treated successfully with placement of additional covered stents. One type IB endoleak from a bell-bottom iliac limb was treated with an iliac branch endoprosthesis. There were 2 instances of in-stent stenosis—1 renal and 1 SMA stent treated with angioplasty and additional stent placement. There were no TA occlusions observed during follow-up surveillance. Three-year Kaplan-Meier estimates of freedom from secondary intervention and freedom from TA instability were 56±11 and 68±11%, respectively (Figure 2B and C). Primary and secondary TA patency rates were 88±8 and 100%, respectively.

3-year Kaplan-Meier estimates of patient survival (A), freedom from secondary intervention (B), and freedom from target artery instability (C) of 22 patients undergoing complete aortic repair.
Discussion
This report describes a single-center experience of complete aortic repair (zones 0-9) performed by a dedicated team with expertise in complex open, endovascular and hybrid aortic surgery. Despite replacement and/or coverage from the ascending aorta to the aortoiliac bifurcation, perioperative and 3-year outcomes described herein are comparable to other studies analyzing isolated thoracic aortic and thoracoabdominal aortic repair. The incidence of SCI was low with one patient (5%) discharged with residual neurologic deficits. Despite the low number of patients, these results support the notion that multiple staged aortic procedures can be performed safely and effectively with open surgical or endovascular ascending and aortic arch repair followed by endovascular repair of the distal thoracoabdominal aorta.14,15,28
As medical therapies improve and life expectancy subsequently increases, patients with complex aortic disease often present at an advanced age with multiple cardiovascular comorbidities that greatly increase their operative risk. These patients are often unfit for complete open repair of the entirety of the diseased thoracic and abdominal aortic segments. In some patients, the physiologic and emotional toll from the index open aortic procedure precludes completion repair. In fact, studies have shown that up to 40% of those who are successfully treated with TAR never undergo the intended distal TAAA reconstruction, leaving them at risk for rupture.29–31 Contemporary 30-day mortality and SCI rates of open surgical TAR with FET range between 8-15% and 3-6%, respectively.1,2 Similarly, series examining modern outcomes of open TAAA repair demonstrate 30-day mortality rates of 7% to 17% and an incidence of SCI of 2% to 14%.3,4 While endovascular repair of complex abdominal and TAAAs has gained widespread acceptance owing to decreased early morbidity and mortality compared to open repair, total endovascular repair of the aortic arch remains limited to select highly specialized centers with access to manufactured endografts. Early and mid-term results of total endovascular arch repair from highly experienced operators demonstrate satisfactory results; however, the broad applicability remains unknown at this time.6,28,32
Several studies have analyzed the role of repair and reconstruction of the entire aorta. Estrera and colleagues reported on 39 patients with a mean age of 53 years old and 39% incidence of genetic aortopathy who underwent 87 staged open aortic reconstructions. Results were impressive with no early mortality or stroke and an 8% (3/39) incidence of paraplegia following distal repair. Survival at 5, 10, and 20 years was 71%, 38%, and 30%, respectively. 33 However, comparison of endovascular and open arch and TAAA repair is difficult, as most large series of open reconstructions include younger patients with less comorbidities and a higher incidence of genetic aortopathy than patients included in series of complex EVAR.1–6 In this study, the mean age was 72 years old with a high incidence of multiple cardiopulmonary risk factors and a 10% incidence of genetic aortopathy (Table 1).
Tsilimparis and colleagues published the 12-month outcomes of 33 patients who underwent endovascular arch and TAAA repair reporting a 6% early mortality and a 3% incidence of both permanent paraplegia and stroke. Survival and freedom from secondary intervention at 12 months were 72% and 82%, respectively. 28 Surgical TAR+FET followed by distal FB-EVAR has been described in 2 studies which include some of the patients included in the present series and demonstrate the need for proper patient selection given the limited 3- and 5-year survival of this patient population.14,15,34
Several lessons have been learned from this experience. First, establishment of a comprehensive aortic team is paramount for the delivery of evidence-based care while practicing informed and shared decision-making. Multidisciplinary teams have been found to improve outcomes in other facets of cardiovascular disease that have both open and endovascular treatment options such as aortic valve disease.35,36 Aortic teams should include cardiologists with expertise in acquired and congenital aortopathies, cardiothoracic anesthesiologists, cardiovascular surgeons, and vascular surgeons. Operative planning is performed using centerline reconstruction of the entire aorta and its branches. Intentional consideration of all possible future aortic interventions, their potential complications and necessary bailouts are of great importance. A consistent operating room team, immediate in-hospital availability of physicians comfortable with placing spinal drains emergently, as well as standardized postoperative care protocols all contribute to reduction of MAEs and improvement in clinical outcomes. A learning curve should be expected with our institution observing a progressive reduction in perioperative morbidity and mortality despite increasing aneurysm complexity. 37
When planning ascending, arch, and proximal descending thoracic aortic repair, the need for future upper extremity endovascular access should be considered. While our preference and practice has shifted to a total femoral approach when safe and feasible, intentional design decisions should be made to allow for antegrade upper extremity endovascular device delivery should it be deemed necessary. Open surgical repair of the arch should include great vessel anastomoses that are 90° in direction to allow for passage of large bore sheaths. Highly angulated anastomoses or carotid-subclavian bypasses may complicate or preclude antegrade device delivery and should be avoided when possible (Figure 3). 38 Ascending aortic and hemiarch replacement should be optimized for future endovascular arch repair, ensuring the ascending graft is long and free of kinks to allow a minimum of 2 cm—but ideally 4 cm—of proximal landing zone. 39

When planning for endovascular intervention of the thoracoabdominal aorta, surgical great vessel anastomoses should be created at 90° (A) to allow for passage of sheaths and catheters via the upper extremity. Acute angulation (B) increases the difficulty of antegrade device delivery. In cases of endovascular arch repair, a retrograde left subclavian artery branch (C) allows for utilization of upper extremity access for thoracoabdominal intervention.
The incidence of SCI—the most dreaded complication of open and endovascular aortic surgery—can be minimized with appropriate device design, procedural staging, protocolized intraoperative and postoperative care pathways, and spinal drain placement. Shorter thoracic endografts used for FET are associated with decreased incidence of SCI and obviate the need for prophylactic spinal drainage—our preference being a straight 10 cm length device when feasible—however, these patients typically require interval TEVAR before completion TAAA repair. 40 Whereas procedural staging has been demonstrated to reduce SCI and improve mortality, the risk of interval aortic rupture must be factored in when determining the appropriate timing between interventions. 41
When using patient-specific endografts, planning and staging must take into account the manufacturing and shipping time required for implantation of these devices. 42 Considering recent data from our group as well as others, we have reduced the time between stages and aim to perform second stage TEVAR before or shortly after hospital discharge following ascending and aortic arch repair. In a series of 120 candidates for staged endovascular repair of TAAAs, 13% experienced interval aortic events at a mean time of 17 days following TEVAR. Maximum aortic diameter was the primary risk factor for adverse events between aneurysm exclusion and endograft deployment beyond the area of maximum aneurysm diameter was not protective. 43 Aside from aortic diameter, toleration of previous interventions and optimization of cardiovascular risk factors are the primary determinants of timing of the completion TAAA repair, with our current preference being 2 to 6 weeks after second stage TEVAR.
With greater understanding and recognition of the efficacy and safety profile of spinal drain placement, our practice has shifted from routine prophylactic to select preoperative and as needed rescue drain insertion. 24 This practice is reliant on immediate availability of physicians capable of placing emergent rescue spinal drains. A multicenter experience including patients from our institution demonstrated repair of extensive TAAAs without prophylactic spinal drains is safe, with a 2% incidence of SCI and clinical improvement in 73% of those receiving rescue drain placement. 44 In the present series, 2 (13%) patients undergoing TAR+FET and 1 (5%) patient undergoing thoracoabdominal FB-EVAR suffered from a spinal drain complications requiring intervention. All patients (4/22; 18%) who incurred SCI had prophylactic spinal drains placed preoperatively.
There are several limitations of this study. First, the partial retrospective methodology and inclusion of several patients who underwent thoracic aortic repair at outside institutions limits the accuracy and granularity of the data collection. Second, while IDE patients who underwent endovascular arch and TAAA repair were followed in a prospective manner, those who received open surgical ascending and arch repair at our institution were not prospectively studied, and therefore, we are unable to account for any patients who were intended or would have eventually required complete aortic repair. Third, all patients were cared for by highly experienced surgeons and perioperative teams which may limit reproducibility at other institutions. Finally, the low number of patients undergoing endovascular arch repair and TAAA repair with PMEGs prevented comparative analysis.
Conclusion
Complete repair of the aorta with open and endovascular techniques is safe and feasible when performed at a high-volume, experienced aortic center with excellent early and mid-term aortic-related outcomes and satisfactory rates of SCI and TA instability. A multidisciplinary team is essential for proper patient selection, perioperative care, and postoperative surveillance.
Footnotes
Author’s Note
CONFERENCE PRESENTATION
Presented at the 49th annual meeting of the VEITH Symposium on November 15-18th, 2022 in New York, NY.
Declaration of Conflicting Interests
The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: GSO receives consulting fees and research grant support from Centerline Biomedical, Cook Medical, G.E. Healthcare, and W.L. Gore. GSO has a Global Principal Investigator agreement with Cook Medical. BCM receives consulting fees and research support from Cook Medical and W.L. Gore and is a regional aortic advisor for Medtronic with all fees paid to Mayo Clinic. All other authors report no conflicts of interest—financial or otherwise.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
