Use of physician-modified inverted limb in conjunction with Zenith fenestrated stent graft to rescue failed previous endovascular and open repair.

OBJECTIVE: To evaluate outcomes and performance of inverted limbs (ILs) when used in conjunction with Zenith fenestrated stent grafts (Zfens) to treat patients with short distance between the lowest renal artery (RA) and aortic or graft bifurcation (A/GB). METHODS: This study was a multicenter, retrospective review of prospectively maintained database of patients with complex aortic aneurysms, failed endovascular aneurysm repair (EVAR), or open surgical repair (OSR) with short distance between LRA and A/GB treated using a combination of Zfen and an IL between 2013 and 2023. Endpoints included technical success, aneurysm sac regression, long-term device integrity, and target vessel patency. We defined technical success as implantation of the device with no endoleak, conversion to an aorto-uni-iliac or OSR. RESULTS: During this time, 52 patients underwent endovascular rescue of failed repair. Twenty (38.5%) of them required relining of the failed repairs using IL due to lowest RA to A/GB length restrictions. Two patients had undergone rescue with a fenestrated cuff alone but developed type III endoleaks. One patient with no previous implant had a short distance between the lowest RA and aortic bifurcation to accommodate the bifurcated distal device, and two patients had failed OSR or anastomotic pseudoaneurysms. The majority (94%) were men with a mean age of 76.8 ± 6.1 years. The mean aortic neck diameter and aneurysm size were 32 ± 4 cm and 7.2 ± 1.3 cm, respectively. The median time laps between initial repair and failure was 36 months (interquartile range [IQR], 24-54 months). Sixteen patients (80%) were classified as American Society of Anesthesiologists class III, whereas four were class IV. Seventy-eight vessels were targeted and successfully incorporated. Technical success was 100%, and median estimated blood loss was 100 mL (IQR, 100-200 mL). Mean fluoroscopy time and dose were 61 ± 18 minutes and 2754 ± 1062 mGy, respectively. Average hospital length of stay was 2.75 ± 2.15 days. Postoperative complication occurred in one patient who required lower extremity fasciotomy for compartment syndrome. At a median follow-up of 50 months (IQR, 18-58 months), there were no device migration, components separation, aneurysmal related mortality, and type I or type III endoleak. Aneurysm sac regression (95%) or stabilization (5%) was observed in all patients, including in four patients (25%) with type II endoleak. CONCLUSIONS: The use of IL in conjunction with Zfen to treat patients with short distance between the lowest RA and A/GB is safe, effective, and has excellent long-term results. The technique expands the indication of Zfen, especially in patients with failed previous EVAR.

Examining the impact of median arcuate ligament-induced celiac artery compression on target vessel patency, long-term survival and device integrity in fenestrated and branched endovascular repairs.

OBJECTIVE: To evaluate the impact of celiac artery (CA) compression by median arcuate ligament (MAL) on technical metrics and long-term CA patency in patients with complex aortic aneurysms undergoing fenestrated/branched endograft repairs (F/B-EVARs). METHODS: Single-center, retrospective review of patients undergoing fenestrated/branched endovascular aortic aneurysm repairs and requiring incorporation of the CA between 2013 and 2023. Patients were divided into two groups-those with (MAL+) and without (MAL-) CA compression-based on preoperative computed tomography angiography findings. MAL was classified in three grades (A, B, and C) based on the degree and length of stenosis. Patients with MAL grade A had ≤50% CA stenosis measuring ≤3 mm in length. Those with grade B had 50% to 80% CA stenosis measuring 3 to 8 mm long, whereas those with grade C had >80% stenosis measuring >8 mm in length. End points included device integrity, CA patency and technical success-defined as successful implantation of the fenestrated/branched device with perfusion of CA and no endoleak. RESULTS: One hundred and eighty patients with complex aortic aneurysms (pararenal, 128; thoracoabdominal, 52) required incorporation of the CA during fenestrated/branched endovascular aortic aneurysm repair. Majority (73%) were male, with a median age of 76 years (interquartile range [IQR], 69-81 years) and aneurysm size of 62 mm (IQR, 57-69 mm). Seventy-eight patients (43%) had MAL+ anatomy, including 33 patients with MAL grade A, 32 with grade B, and 13 with grade C compression. The median length of CA stenosis was 7.0 mm (IQR, 5.0-10.0 mm). CA was incorporated using fenestrations in 177 (98%) patients. Increased complexity led to failure in CA bridging stent placement in four MAL+ patients, but completion angiography showed CA perfusion and no endoleak, accounting for a technical success of 100%. MAL+ patients were more likely to require bare metal stenting in addition to covered stents (P = .004). Estimated blood loss, median operating room time, contrast volume, fluoroscopy dose and time were higher (P < .001) in MAL+ group. Thirty-day mortality was 3.3%, higher (5.1%) in MAL+ patients compared with MAL- patients (2.0 %). At a median follow-up of 770 days (IQR, 198-1525 days), endograft integrity was observed in all patients and CA events-kinking (n = 7), thrombosis (n = 1) and endoleak (n = 2) -occurred in 10 patients (5.6%). However, only two patients required reinterventions. MAL+ patients had overall lower long-term survival. CONCLUSIONS: CA compression by MAL is a predictor of increased procedural complexity during fenestrated/branched device implantation. However, technical success, long-term device integrity and CA patency are similar to that of patients with MAL- anatomy.

Impact of continued clopidogrel use on outcomes of patients undergoing carotid endarterectomy.

OBJECTIVE: The aim of this study was to evaluate the use of clopidogrel at the time of carotid endarterectomy (CEA) and its association with postoperative complications. METHODS: Single-institution, retrospective review of a prospective database. RESULTS: From 2010 to 2017, CEA was performed in 1066 consecutive patients (median age, 73 years; 66% men). The indications for operation included ≥70% asymptomatic stenosis (458; 43%), prior stroke (314; 29%), and transient cerebral or retinal ischemia (294; 28%). At the time of operation, 509 (48%) patients were taking aspirin alone, 441 (41%) were taking clopidogrel (374 in combination with aspirin, 67 as sole therapy), 83 (8%) were on no documented antiplatelet medication, and 33 (3%) were taking warfarin (with therapeutic international normalized ratio). The likelihood of clopidogrel use at the time of operation was higher for patients with a history of symptomatic carotid disease (P = .002). Over the study period, clopidogrel use increased from 31.9% in 2010 to 56.8% in 2017, which corresponds to an 11% (95% confidence interval, 6%-15%) increase annually. Postoperative strokes occurred in 15 patients (overall incidence, 1.4%), the majority of which were minor (12/15; 80%). Six strokes occurred in patients taking aspirin alone (6/509; 1.2%), two in patients on clopidogrel and aspirin (2/441; 0.5%), two in patients taking clopidogrel alone (2/67; 2.9%), three in patients on no documented antiplatelet medication (3/83; 3.6%), and two in those taking warfarin (one of which was secondary to a fatal intracranial hemorrhage within 30 days of discharge [2/33; 6.1%]). The 30-day mortality rate was 0.03% (3/1066); the risk for the combined endpoint of any stroke, death, or myocardial infarction (MI) was 2.3% (25/1066), and the risk for major stroke, death, or MI was 1.2%. There was no apparent association between clopidogrel use and the incidence of postoperative bleeding (P = .59) or any other postoperative complication (stroke, death, MI, cranial nerve injury; P = .15). CONCLUSIONS: Clopidogrel use in our CEA practice has increased over time and has not been associated with an increased risk of postoperative complications, including bleeding. These data suggest that clopidogrel should not be discontinued prior to CEA and should be considered as part of 'optimal medical therapy' in patients undergoing CEA.

Effects of Iliac Tortuosity Index on Fenestrated Endovascular Aortic Aneurysm Repair for Pararenal and Thoracoabdominal Aortic Aneurysms.

PURPOSE: To evaluate the effect of iliac tortuosity on procedural metrics and outcomes of patients with complex aortic aneurysms (cAAs) undergoing repair with fenestrated/branched endografts (f/b-EVAR [endovascular aortic aneurysm repair]). MATERIAL AND METHODS: The study is a single-center, retrospective review of a prospectively maintained database of patients undergoing aneurysm repair using f/b-EVAR between the years 2013 and 2020 at our institution. Included patients had at least 1 preoperative computed tomography angiography (CTA) available for analysis. Iliac artery tortuosity index (TI) was calculated using centerline of flow imaging from a 3-dimensional work station based on the formula: (centerline iliac artery length / straight-line iliac artery length). The associations between iliac artery tortuosity and procedural metrics, including total operative time, fluoroscopy time, radiation dose, contrast volume, and estimated blood loss (EBL), were evaluated. RESULTS: During this period, 219 patients with cAAs underwent f/b-EVAR at our institution. Ninety-one patients (74% men; mean age = 75.2±7.7 years) met criteria for inclusion into the study. In this group, there were 72 (79%) juxtarenal or paravisceral aneurysms and 18 (20%) thoracoabdominal aortic aneurysms and 5 patients (5.4%) with failed previous EVAR. The average aneurysm diameter was 60.1±0.74 mm. Overall, 270 vessels were targeted, and 267 (99%) were successfully incorporated, including 25 celiac arteries, 67 superior mesenteric arteries, and 175 renal arteries. The mean total operative time was 236±83 minutes, fluoroscopy time was 87±39 minutes, contrast volume was 81±47 mL, radiation dose 3246±2207 mGy, and EBL was 290±409 mL. The average left and right TIs for all patients were 1.5±0.3 and 1.4±0.3, respectively. On multivariable analysis, the interval estimates suggest positive association between TI and procedural metrics to a certain degree. CONCLUSIONS: In the current series, we found no definitive association between iliac artery TI and procedural metrics, including operative time, contrast used, EBL, fluoroscopy time, and dose in patients undergoing cAA repair using f/b-EVAR. However, there was a trend toward association between TI and all these metrics on multivariable analysis. This potential association needs to be evaluated in a larger series. CLINICAL IMPACT: Iliac artery tortuosity should not exclude patients with complex aortic aneurysms from being offered fenestrated or branched stent graft repair. However, special considerations should be taken to mitigate the impact of access tortuosity on alignment of fenestrations with target vessels, including use of extra stiff wires, through and through access and delivering the fenestrated/branched device into another (larger) sheath such as a Gore DrySeal in patients with arteries large enough to accommodate such sheaths.

Spontaneous iliac vein rupture and thrombosis in patients with May-Thurner syndrome: A narrative review.

Spontaneous iliac vein rupture (SIVR) is extremely rare and can lead to serious complications, including death. Etiologies include inflammatory processes and hormonal and mechanical triggers, with concomitant May-Thurner syndrome (MTS) being a rare cause. Management can be challenging due to the difficult balance between reducing thrombotic burden and life-threatening hemorrhage that can result from aggressive anticoagulation. Furthermore, surgical interventions are associated with high mortality, making conservative management more desirable. We report a case of SIVR with retroperitoneal hematoma and concurrent MTS that was successfully managed using conservative measures. We further provide a narrative review of the current literature addressing the diagnosis, management, and outcome of SIVR focusing on cases with concurrent MTS.

Effects of SGLT2 inhibitors and GLP1-receptor agonists on cardiovascular and limb events in peripheral artery disease: A review.

Peripheral artery disease (PAD) and diabetes mellitus are two overwhelming health problems associated with major cardiovascular (CV) and limb events, in addition to increased mortality, despite advances in medical therapies including statins and renin-angiotensin system inhibitors. Sodium-glucose cotransporter 2 inhibitors (SGLT2i) and glucagon-like peptide 1-receptor agonists (GLP1-RA) are two new antihyperglycemic drug classes that have been associated with a significant reduction of major adverse cardiovascular events (MACE) in patients with type 2 diabetes (T2D) and CV risk. Whereas most studies had enrolled patients with T2D and concurrent CV disease (CVD), patients with PAD were obviously underrepresented. Furthermore, there was a signal of increased risk of amputation in one of the main trials with canagliflozin. We aim to provide a general review of the current literature and summarize societal guideline recommendations addressing the role of SGLT2i and GLP1-RA drugs in patients with CVD focusing on the PAD population when data are available. Endpoints of interest were MACE and, when available, major adverse limb events (MALE).

Outcomes in patients discharged with extended venous thromboembolism prophylaxis after hospitalization with COVID-19.

BACKGROUND: Venous thromboembolism (VTE) is a known complication of coronavirus disease (COVID-19) in patients requiring hospitalization and intensive care. We examined the association between extended pharmacological VTE prophylaxis and outcomes among patients hospitalized with COVID-19. METHODS: This was a retrospective cohort study of patients with an index positive SARS-CoV-2 polymerase chain reaction (PCR) test at the time of, or during hospitalization. Patients who were prescribed extended pharmacological VTE prophylaxis were compared against patients who were not. Multivariable logistic regression was used to produce odds ratio (OR) estimates and Cox proportional hazard models for hazard ratios (HR) with 95% CI to examine the association between pharmacological VTE prophylaxis and outcomes of interest. Primary outcomes were 30- and 90-day VTE events. Secondary outcomes included 30- and 90-day mortality, 30-day superficial venous thrombosis (SVT), acute myocardial infarction (MI), acute ischemic stroke, critical limb ischemia, clinically significant bleeding, and inpatient readmissions. RESULTS: A total of 1936 patients were included in the study. Among them, 731 (38%) were discharged on extended pharmacological VTE prophylaxis. No significant difference was found in 30- and 90-day VTE events among groups. Patients discharged on extended VTE prophylaxis showed improved survival at 30 (HR: 0.35; 95% CI: 0.21-0.59) and 90 days (HR: 0.36; 95% CI: 0.23-0.55) and reduced inpatient readmission at 30 days (OR: 0.12; 95% CI: 0.04-0.33) when compared to those without. CONCLUSION: Patients discharged on extended VTE prophylaxis after hospitalization due to COVID-19 had similar thrombotic events on follow-up. However, use of extended VTE prophylaxis was associated with improved 30- and 90-day survival and reduced risk of 30-day inpatient readmission.

Impact of a multidisciplinary acute aortic dissection program: Improved outcomes with a comprehensive initial surgical repair strategy.

OBJECTIVE: As part of a multidisciplinary aortic dissection (AD) program, a more comprehensive repair strategy for patients with acute type A aortic dissection (ATAAD) and frequent endografting for suitable patients with type B aortic dissection (ATBAD) was adopted in 2015. The aim of this study was to evaluate the impact of these changes. METHODS: This study is a retrospective review of a prospective database containing all patients treated for acute AD between 2003 and 2020. Patients were grouped based on differing repair strategies (pre 2015 vs post 2015). Clinical characteristics, procedural details, and survival data were analyzed. RESULTS: During this time, 323 patients (210 pre, 113 post) were treated for acute AD at our institution. There were 221 patients with ATAAD (149 pre, 72 post) and 102 patients with ATBAD (61 pre, 41 post). The majority (60%) were males, with a mean age of 65.9 ± 15.2 years. There were no differences in cardiovascular risk factors or demographics between the groups. After 2015, fewer patients with ATAAD underwent medical management alone (15% pre vs 4% post; P = .014), and most that underwent surgical intervention had a total arch or aggressive hemiarch repair (27% pre vs 78% post; P < .001). Seventy-four patients (73%) with ATBAD were treated medically, whereas 28 underwent medical management and endografting (23% pre, 34% post; P = .214). For all patients with AD, 30-day mortality was significantly improved (26% pre vs 10% post; P < .001) especially among patients who underwent ATAAD surgery (23% pre vs 9% post; P = .018). Three-year Kaplan-Meier survival estimates showed survival improvement among patients with ATAAD (Log rank P-value = .019); however, this improvement does not extend to type B dissections or the overall cohort. A survival analysis landmarked to 30 days after initial presentation showed no statistical difference in survival from 30 days to 3 years post-presentation. CONCLUSIONS: A more comprehensive repair strategy in the management of patients with acute AD resulted in improved overall patient outcomes and significantly decreased 30-day mortality, even though more complex repairs were performed. The long-term impact of the changes made to our program remains to be evaluated.

Technical approach, outcomes, and exposure-related complications in patients undergoing anterior lumbar interbody fusion.

OBJECTIVE: To describe our technique, evaluate access related complications and factors contributing to adverse outcomes in patients undergoing retroperitoneal anterior lumbar interbody fusion (ALIF). METHODS: We conducted a retrospective analysis of prospectively collected data on patients undergoing ALIF at our institution from January 2008 to December 2017. Access was performed by a vascular surgeon who remained present for the duration of the case. Data collected included patients' demographics, comorbidities, exposure related complications and ileus. Study end points included major adverse events and minor complications. Major adverse events included any vascular injuries requiring repair, bowel and ureter injuries, postoperative bleeding requiring reoperation, myocardial infarction, stroke, venous thromboembolism (pulmonary embolism/deep venous thrombosis), wound dehiscence, and death. Minor complications included postoperative paralytic ileus, urinary tract infections, and surgical site infections. The incidence of incisional hernia was also evaluated. RESULTS: During this period, 1178 patients (514 males and 664 females; mean age, 54.1 ± 13.8 years) underwent a total of 2352 levels ALIF at our institution (single level, 422 patients; 2 levels, 450; 3 levels, 205; 4 levels, 98; 5 levels, 6; 6 levels, 1; and 7 levels, 1). The median estimated blood loss was 25 mL (interquartile range, 25-50). There were 57 exposure-related complications (4.8%), including vascular injuries (venous, 13; arterial, 4) in 17 patients (1.4%), bowel injuries in three patients (serosa tear in two and arterial embolization with subsequent bowel ischemia in one). Eleven of the 13 venous injuries (84.6%) occurred while exposing the L4 to L5 lumbar level. Two of the four patients with arterial injuries developed acute limb ischemia requiring embolectomy. One embolized to the superior mesenteric artery and underwent bowel resection. Twenty patients (1.7%) developed venous thromboembolism, two of whom had sustained left iliac vein injury during exposure. Sixteen patients (1.4%) developed a retroperitoneal hematoma/seroma with nine requiring evacuation in the operating room. Thirty-six patients (3.1%) developed postoperative ileus, defined as an inability to tolerate diet on postoperative day 3. Four patients (0.4%) had a postoperative myocardial infarction, and two had a stroke and two (0.17%) died within the first 30 postoperative days. Thirty-one patients developed incisional complications, including surgical site infection in 24 and incisional hernia in 7. CONCLUSIONS: Our findings suggest that ALIF exposure can be performed safely with a relatively low overall complication rate. The majority of vascular injuries associated with this procedure are venous in nature, occurring predominantly while exposing the L4 to L5 level and can be safely addressed by an experienced vascular team.

Lipid-lowering therapies in peripheral artery disease: A review.

Peripheral artery disease (PAD) is estimated to affect approximately 8.5 million individuals in the US above the age of 40, and is associated with significant morbidity, mortality, and impairment. Despite the significant adverse limb and cardiovascular (CV) outcomes seen in patients with PAD, there is typically less attention paid to risk factor modification relative to other atherosclerotic diseases such as coronary artery disease (CAD) or stroke. In the current literature, statins have been shown to reduce mortality, major adverse CV events, major adverse limb events, and improve symptomatic outcomes in patients with PAD. In addition, proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors are emerging as an additional lipid-lowering therapy for patients with PAD. However, despite current guideline recommendations based on growing evidence, patients with PAD are consistently undertreated with lipid-lowering therapies. We provide an extensive literature review and evidence-based recommendations for the use of statins and PCSK9 inhibitors in patients with PAD.

Risk and Management of Venous Thromboembolism in Patients with COVID-19.

BACKGROUND: One of the most pronounced and poorly understood pathological features of COVID-19 infection has been high risk for venous and arterial thromboembolic complications. An increasing number of thromboembolic events are being reported almost on a daily basis, and the medical community has struggled to predict and mitigate this risk. We aimed to review available literature on the risk and management of COVID-19 related venous thromboembolism (VTE), and provide evidence-based guidance to manage these events. METHODS: A literature review of VTE complications in patients with COVID-19 was performed, in addition to a summary of the societal guidelines and present pathways implemented at our institution for the management of both in- and outpatient COVID-19 related VTE. RESULTS: Although a significant VTE risk has been confirmed in patients with COVID-19, literature addressing best ways to mitigate this risk is lacking. Furthermore, there has been very limited guidance provided by societal guidelines to help prevent and manage VTE associated with the COVID-19 infection. In light of the available data, we advise that all patients admitted with suspected or confirmed COVID-19 receive pharmacological prophylaxis if bleeding risk is acceptable. For patients with COVID-19 who have been discharged from the emergency department or hospital, we suggest extended thromboprophylaxis (up to 39 days) as long as bleeding risk is low. CONCLUSIONS: We believe that this literature summary along with our center recommendations and algorithms provide valuable guidance to providers caring for patients with COVID-19 related VTE. More research is needed to standardize prophylaxis and management protocols for these patients.

Determining Risk Factors for Endovenous Heat-induced Thrombosis after Radiofrequency Ablation.

BACKGROUND: The objective of this study was to evaluate risk factors, incidence, management, and outcome of endovenous heat-induced thrombosis (EHIT) related to radiofrequency ablation (RFA). METHODS: This was a single-center retrospective analysis of patients over the age of 18 who underwent RFA between 2016 and 2019. Demographics, comorbidities, medications, severity scores, vascular anatomy, procedural details, and outcome data were collected. EHIT-related data included occurrence, grade, laterality, management, and outcome. RESULTS: During the study period, 672 RFA procedures were performed at our institution. Of these, 642 (median age 57 (21-93), 62.3% female) met study inclusion criteria. EHIT was observed in 43 (6.6%) cases. Concurrent left common femoral vein (CFV) or right femoral vein (FV) incompetence was found to be more prevalent in the EHIT group (P = 0.024 and P = 0.011, respectively). Compared with performing RFA alone, concurrent performance of stab phlebectomy and sclerotherapy with RFA on the left side was found to be associated with possible increased risk for EHIT (P = 0.021). Furthermore, patients with diabetes mellitus (DM) (P = 0.05) and those with median diameter of the treated left vein of 1.2 cm (P = 0.02) were more likely to have a higher EHIT grade (III and IV) than those without DM and those with smaller vein diameter, respectively. Management included aspirin (44%), anticoagulant (28%), both (10%), or neither (18%). EHIT either resolved or regressed (64%), did not change (5%), or propagated (8%) at follow-up. CONCLUSIONS: Left CFV or right FV incompetence was found to be more prevalent in the total EHIT group. Furthermore, DM and the median size of the treated vein on the left (1.2 cm) were more prevalent in the high (III-IV) versus low grade (I-II) EHIT group. More than 50% of EHIT improved (regressed or resolved) at follow-up regardless of the management option. Further analysis with larger patient samples are needed to confirm the association between these variables and the development of EHIT.

Type A Aortic Dissection Caused by Loeys-Dietz Syndrome with Novel Variation.

Loeys-Dietz syndrome (LDS) is a rare autosomal-dominant connective tissue disorder that can lead to aortic aneurysm and dissection. There are 5 different types caused by mutations in TGFßR1 (transforming growth factor ß receptor), TGFßR2, SMAD3, TGFß2 (transforming growth factor ß), and TGFß3 respectively. The prevalence of LDS is estimated to be less than 1 in 100,000. There is considerable variability in the phenotype of LDS, from mild features to severe systemic abnormalities. There is overlap in the manifestations of LDS and Marfan syndrome, including increased risk of ascending aortic aneurysm and aortic dissection, as well as abnormally long limbs and fingers. Management can be very challenging with a high risk of complications with revascularization. We report a 60-year-old female who presented with a type A aortic dissection that originated from the aortic root and extended to the bilateral common femoral arteries. Genetic testing revealed a novel alteration of the TGFßR1 gene (c689 C>A in exon 4) that to our knowledge has not been previously reported or found in large population cohorts. She was managed through a Bentall procedure that was complicated by a graft tear and stenosis of the distal anastomosis site, in addition to requiring a temporary pacemaker implantation and hemodialysis after the procedure. Ultimately, the patient was able to recover fully.

Comparative Long-term Outcomes of Patients with Aortoiliac Occlusive Disease Limited to Common Iliac Arteries Who Underwent Endarterectomy versus Bypass Grafting.

BACKGROUND: The objective of this study was to compared outcomes of patients with aortoiliac occlusive disease (AIOD), limited to the common iliac artery, who underwent either aortoiliac thromboendarterectomy (AIE) or aortobiiliac bypass grafting (ABIB). METHODS: A single-center, retrospective analysis of consecutive patients with AIOD who underwent either AIE or ABIB between 2010 and 2019 from a prospective database. Patients with disease extending to the external iliac or common femoral arteries were excluded. Data collected included demographics, cardiovascular risk factors, indication for surgery, preoperative and postoperative ankle brachial indexes (ABIs), estimated blood loss, major adverse events (MAEs), and long-term patency. The study end point was clinical success, defined as improvement in ABIs with resolution of symptoms. MAEs included return to the operating room for any reason, postoperative myocardial infarction, stroke, pneumonia, or venous thromboembolism. RESULTS: Thirty-three patients, who met inclusion criteria, underwent repair for AIOD (AIE: 13; ABIB: 20) at our institution during this time. In both groups, there were more women than men (AIE: 11, ABIB: 10) with a mean age of 55 ± 7 years and 58 ± 6 years in the AIE and ABIB group, respectively. Indication for surgery included disabling claudication in 19 patients, ischemic rest pain in 13 patients, and tissue loss in one patient. No difference in cardiovascular risk factors or AIOD severity was noted between groups. Patients in the AIE group had slightly higher body mass index (30 ± 5 vs. 26 ± 6, P = 0.06). Two patients in each group required concomitant renal/mesenteric artery endarterectomy. One patient in the AIE group required bilateral femoral artery exposure and external iliac thrombectomy. MAEs (4 vs. 0) were higher in the ABIB group including, pneumonia in one patient, myocardial infarction in another, return to the operating room for evacuation of hematoma in the third and bypass graft thrombectomy with lower extremity angiography in the fourth patient. There were no differences in the intensive care unit or hospital length of stay between groups. Patients in both groups achieved return of normal ABI and complete resolution of their symptoms. At mean follow-up time of 43.4 ± 25.2 and 52.9 ± 35.4 months in the AIE and ABIB group, respectively, there was no symptomatic recurrence or need for reintervention while two patients in the ABIB group died of non-aortic-related issues. CONCLUSIONS: Both procedures were safe, effective, and conferred high long-term primary patency with no need for reintervention in patients with AIOD limited to the common iliac arteries.

Digital ischemia in Behçet's disease: case-based review.

Behçet's disease is a rare chronic autoimmune disease affecting primarily Middle and East Asian populations between the ages of 20 and 40 years. Behçet's disease manifests with oral and genital mucocutaneous lesions, ocular disease, venous thrombosis, and central nervous system degradation. Treatment can be challenging and may require immunosuppressive agents and/or topical wound-care. While larger vascular involvement has been reported, digital ischemia due to small-vessel involvement has not been well described in the literature. Based on a systematic literature review, we were only able to find seven published cases of limb ischemia, none of which reported digital involvement. We present a unique case of Behçet's disease with severe digital ischemia and ulceration caused by small-vessel involvement. The patient was managed successfully with antiplatelet, immunosuppressants, and anti-inflammatories with complete resolution of the ischemic symptoms. By focusing on small-vessel involvement and digital ischemia, we provide insight into clinical presentation and treatment for this very rare vascular manifestation of Behçet's disease.

Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases.

Segmental arterial mediolysis (SAM) is a rare but serious nonatherosclerotic, noninflammatory vasculopathy of unknown etiology that often results in dissection, aneurysm, occlusion, or stenosis of, primarily, the abdominal arteries. Current literature lacks consensus on diagnostic criteria and management options for SAM. This review summarizes 143 cases and aims to advance appropriate recognition and management of SAM. Literature review of all relevant SAM case studies from 2005 to 2018 yielded 126 individual SAM cases from 66 reports. We identified 17 additional SAM cases from our center, bringing our analysis to 143 patients. Patients with SAM were most commonly men (68%) in their 60s. Hypertension (43%), tobacco use (12%), and hyperlipidemia (12%) were common comorbidities. Abdominal pain (80%) and intraabdominal bleeding (50%) were the most common presenting symptoms. Computed tomography was the most frequently used imaging method (78%), and histology was available in 44% of cases. The most commonly affected vessels were the superior mesenteric (53%), hepatic (45%), celiac (36%), renal (26%), and splenic (25%) arteries with aneurysm (76%), dissection (61%), and arterial rupture (46%). Treatments included coil embolization (28%), abdominal organ surgery (24%), open arterial repair (21%), and medical management (20%). Case-specific treatment modalities yielded symptom relief in the vast majority (91%) of patients, with a mortality rate of 7%.

A Novel Frameshift COL3A1 Variant in Vascular Ehlers-Danlos Syndrome.

Ehlers-Danlos syndromes (EDSs) are a group of heritable connective tissue disorders with distinct genetic etiologies. Of the 13 currently recognized types of EDS, the vascular type EDS (vEDS) is generally considered the most severe and is associated with a decreased life expectancy due to spontaneous arterial, intestinal, and or uterine rupture. Diagnosis of vEDS is supported by genetic testing confirming the presence of pathogenic variations in COL3A1, a type III procollagen gene. Management of vEDS is usually conservative with control of hemodynamic stress, frequent cardiovascular imaging, and, if indicated, a thoughtful endovascular intervention or surgical repair. We present a novel frameshift variant in COL3A1 leading to vEDS with multiple vascular involvements. Based on our literature review, this variant has not been reported and may result in a less severe form of vEDS. Our case report provides insight into genetic variants and clinical expression of vEDS.

Nonatherosclerotic Abdominal Vasculopathies.

BACKGROUND: Nonatherosclerotic abdominal arterial vasculopathies (NAVs), including mesenteric or renal artery dissection, aneurysm, stenosis, and vasculitis, are rare but have great clinical significance. Patients may present emergently with life-threatening complications such as arterial rupture and hemorrhagic shock. Herein, we present our center's experience with NAVs and provide extensive literature review to close the gap in the scarce, related literature. METHODS: From a single-center retrospective data analysis, we identified and characterized subjects (aged 18-60 years) who presented with NAV between January 2000 and December 2015. Of the 1416 charts reviewed, 118 met inclusion criteria. RESULTS: The average age of patients with NAV was 47.0 ± 9.9 years, mostly affecting women (64%). Primary diagnoses included fibromuscular dysplasia (FMD) (25.4%), isolated aneurysms (24.6%), and median arcuate ligament syndrome (MALS) (15.3%). Less common diagnoses were localized vasculitis of the gastrointestinal tract (LVGT) (7.6%), isolated dissection (5.1%), microscopic polyangiitis and granulomatosis with polyangiitis (5.1%), trauma (4.2%), segmental arterial mediolysis (4.2%), Ehlers-Danlos syndrome (2.5%), Takayasu's arteritis (2.5%), polyarteritis nodosa (1.7%), idiopathic abdominal aortitis (0.8%), and Loeys-Dietz syndrome (0.8%). Females constituted 90% of patients with FMD, 77.8% with MALS, 77.8% with isolated aneurysms, 66.7% with Takayasu arteritis, and 55.6% with LVGT. Prevalent comorbidities included tobacco use (43.6%) and hypertension (52.1%). Coil embolization was used in 14.4%, anticoagulation in 11.9%, angioplasty/stenting in 11.9%, open resection/surgical revascularization in 10.2%, and prednisone in 10.2% of the cases. Conservative management was pursued in 33.1% of the patients. A high degree of symptom relief was shown in 91.7%. CONCLUSIONS: NAV are rare and can be caused by different etiologies that primarily affect females. Hypertension and tobacco use were prevalent. Various imaging strategies revealed aneurysms, stenosis, dissection, and/or thrombosis affecting renal and celiac arteries. Most patients improved with conservative, medical, endovascular, or surgical approach. More research is needed to standardize management approach to patients with NAV.

Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review.

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a systemic necrotizing small vessel vasculitis primarily affecting elderly patients. Neutrophil apoptosis and release of pro-inflammatory mediators promote small vessel inflammation and hence multi-organ disease. It rarely affects larger vessels with extremely rare aortic involvement. Diagnosis is made based on clinical presentation, tissue biopsy of affected organ, as well as immunofluorescence and ELISA assays for ANCA. Management includes immunosuppression (e.g., glucocorticoids, cyclophosphamide and rituximab) and supportive therapy. We present a rare case of a younger patient with AAV involving the aorta. The patient's diagnosis was supported by clinical presentation, systemic organ involvement, strongly positive c-ANCA, and skin as well as aortic tissue biopsy results. After failing multiple immunosuppressants, he responded well to rituximab with improved symptoms, inflammatory markers, and imaging findings. Based on our literature review, we were only able to find ten cases of ANCA-related vasculitis involving the aorta. This is the first reported case of successful treatment of AAV-related aortitis using rituximab. Our case report and literature review provide insight into treatment of severe cases of AAV with aortic involvement.

Single-center experience with complex abdominal aortic aneurysms treated by open or endovascular repair using fenestrated/branched endografts.

OBJECTIVE: The objective of this study was to evaluate outcomes of patients with complex abdominal aortic aneurysms (cAAAs) treated with open repair (OR) or fenestrated/branched endovascular aneurysm repair (F/B-EVAR) from a single center. METHODS: A retrospective analysis of consecutive patients with cAAAs treated electively by OR or F/B-EVAR between January 2010 and February 2017 was conducted. Demographics of the patients, cardiovascular risk factors, procedure time, number of vessels incorporated, radiation dose, estimated blood loss, intensive care unit (ICU) length of stay (LOS), and hospital LOS were recorded. End points included target vessel patency, aneurysm rupture, freedom from reintervention, and major adverse events (MAEs). RESULTS: During this period, 153 patients (OR, 69; F/B-EVAR, 84) underwent repair of cAAA. The majority were male (OR, 55; F/B-EVAR, 64), with a mean age of 75.8 ± 7.6 years (F/B-EVAR) and 71.2 ± 7.9 years (OR). Patients in the F/B-EVAR group were more likely to be American Society of Anesthesiologists class 3 and 4 (60% vs 0%; P < .001) and had a higher median Society for Vascular Surgery/American Association for Vascular Surgery comorbidity severity score (15 vs 7; P < .001). A total of 235 vessels were targeted in the F/B-EVAR group, with a technical success of 97.6%. Thirty-one patients in the OR group required concomitant renal artery revascularization. Transfusion requirements (100% vs 1.2%), MAEs (40.6% vs 13.1%), procedure length (304 minutes vs 140 minutes), estimated blood loss (2246 mL vs 165 mL), ICU LOS (3 days vs 1 day), and hospital LOS (7 days vs 2 days) were higher (P < .001) in the OR group compared with the F/B-EVAR group. The 30-day mortality was 2.9% and 2.4% (P = .84) in the OR group and F/B-EVAR group, respectively. Supraceliac clamp site was associated with increased incidence of postoperative renal insufficiency. A decrease in procedure time, contrast volume, fluoroscopy time, and fluoroscopy dose was noted in the F/B-EVAR group with increasing experience even as case complexity increased. More patients were discharged home after F/B-EVAR (97.6% vs 59.4%; P < .001). With a mean follow-up of 31 months (F/B-EVAR, 17 months; OR, 48 months), the rate of secondary intervention was 3.7% and 5.8% (P = NS) for F/B-EVAR and OR, respectively. Freedom from branch instability and reintervention was 99% (95% confidence interval, 96.2%-99.8%) and 96% (95% confidence interval, 87.1%-98.6%), respectively. CONCLUSIONS: Results of this "real-world" experience suggest that the use of F/B-EVAR for the treatment of cAAAs in high-risk surgical patients is safe and effective and has comparable short-term results to those of low-risk patients undergoing OR. Patients treated by F/B-EVAR had shorter ICU and hospital LOS, lower MAEs, and faster convalescence. A decrease in procedure time and radiation dose was noted as experience was gained, even as complexity increased.