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This is a case of a 4-cm left extrathoracic subclavian artery aneurysm (SCAA) in a 58-year-old man with an aortic root and abdominal aortic aneurysm. The patient had features suggestive of genetic arteriopathy, including vertebral artery tortuosity, pectus excavatum, tall stature, and scoliosis. The SCAA was successfully repaired with an inline prosthetic graft and anastomotic pledgets via a supraclavicular approach. Genetic testing revealed an FBN1 pathogenic variant consistent with Marfan syndrome. Repair is satisfactory 2 years later. Patients with SCAA should include consideration of genetic arteriopathy. Open repair of the extrathoracic SCAA in Marfan syndrome is recommended.

To investigate clinical course of patients with type B aortic dissection (TBAD) occurring at a young age with confirmed or suspected heritable thoracic aortic disease.Individuals with TBAD occurring at an age50 years enrolled in the National Registry of the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions Consortium were selected for analysis. Three cohorts were compared: Marfan syndrome (MFS TBAD), nonsyndromic familial TBAD (FTBAD), and sporadic TBAD. Demographics, comorbidities, aortic dissection details, and repair were compared.A total of 150 individuals met inclusion criteria (mean age at TBAD, 36.9 ± 9 years): 73 MFS TBAD, 42 FTBAD, and 35 sporadic TBAD. The cohort of sporadic TBAD had more male patients (71.4%) and fewer individuals of European descent (51.4%) compared with MFS TBAD (57.5% male, 84.9% European descent) and FTBAD (59.5% male, 90.5% European descent). There was a stepwise increase in hypertension prevalence across the cohorts (28.8% MFS, 59.5% FTBAD, 71.4% sporadic TBAD, P.001). Repair of the descending thoracic aorta was performed in 92 cases (67.1% in MFS, 61.9% in FTBAD, and 48.6% sporadic TBAD, P = .18) at a mean of 3.4 ± 5.4 years from TBAD. The repair extent varied. The largest extent of repair was in MFS TBAD, in which thoracoabdominal aortic aneurysm repair was performed in 56.2% compared with 35.7% FTBAD and 17.1% sporadic TBAD (P.001).Control of hypertension is an essential component of care to decrease the risk of TBAD. Over half of the young individuals with TBAD require aortic repair, and individuals with MFS undergo a larger anatomical extent of repair after TBAD.

This analysis of death certificate data in Washington State revealed that age-standardized abdominal aortic aneurysm (AAA) related mortality has declined from 1996 to 2016 with a notable significant drop in ruptured AAA-related mortality. The death rate trends vary by sex, race, and county. OBJECTIVE: Management of abdominal aortic aneurysms (AAA) has undergone considerable advances over the last two decades. Our aim was to evaluate AAA-related mortality trends in Washington State (WA) over a 21 year period and to assess variation in AAA-related mortality by sex, race, and county over the same time period. We hypothesized that a significant decline in AAA-related mortality in WA would be noted. METHODS: Death certificate records were obtained from the WA Department of Health for the period of 1996 to 2016. Records in which AAA was listed as an underlying or an associated cause of death were selected for analysis. Age-standardized mortality rates for each year were calculated using the 2016 WA population as the standard. Mortality trends were compared by sex and race using linear regression. County-specific age-standardized ruptured AAA (rAAA) mortality rates were compared using a Kruskal-Wallis test. RESULTS: Of the 1,014,039 deaths occurring in WA during the study period, 4,438 (0.4%) had AAA listed as an underlying or associated cause of death (66.1% Male, 94.8% White, mean age of death 79.4 ± 9.3 years). In 64.1% of the cases, AAA was listed as the underlying cause of death. AAA-related mortality rates declined by 62.1% over the 21 years from 5.8 2.2 deaths per 100,000. Notably, there was a statistically significant drop in ruptured AAA (rAAA) related mortality rates (3.2 to 0.95 per 100,000, decline of 0.12 death/100,000/year, 95% CI: 0.11–0.14, r(2) = 0.95). Men had a significantly steeper decline in age standardized AAA-related mortality rates with 55% decline (6.5 to 3.0 per 100,000) vs. a 41% decline (2.4 to 1.4 per 100,000) among women. , and were younger at the time of death compared to women (78.1 ± 9.4 vs. 81.9 ± 8.6 years respectively, P

The benefit for carotid endarterectomy (CEA) to prevent a potential stroke has been shown to be less beneficial for women compared with men and the risk of carotid stenting (CAS) is higher in women than men. We hypothesized that a community-based Washington state registry data would also reveal increased morbidity and mortality for women undergoing carotid interventions.Deidentified data for CEA and CAS between 2010 and 2015 were obtained from 19 hospitals participating in the Washington State Vascular-Interventional Surgical Care and Outcomes Assessment Program. Data analysis compared in-hospital composite outcome of stroke and mortality from CEA and CAS between women and men.Over the study period, 3704 individuals underwent CEA (n = 2759; 49.5% symptomatic) and CAS (n = 945; 60.9% symptomatic). Women accounted for 39.5% of the cohort. Women were slightly younger than men (70.0 ± 10.2 vs 71.0 ± 9.6 years respectively; P .01), less likely to be smokers (70.1% vs 75.6%; P .01), and less likely to have a diagnosis of coronary artery disease (32.9% vs 46.5%; P .01). Fewer women underwent CEA for symptomatic carotid disease (46.1% vs 51.8%; P .01). There were no statistically significant differences in the postoperative in-hospital stroke and mortality among women and men undergoing CEA (asymptomatic, 0.8% vs 1.4% [P = .36]; symptomatic, 1.8% vs 2.2% [P = .58]) and CAS (asymptomatic, 1.4% vs 2.2% [P = .56]; symptomatic, 4.6% vs 2.5% [P = .18]). Hospital duration of stay and discharge disposition were similar for women and men. A subanalysis of the octogenarian cohort undergoing CAS demonstrated a substantial increase in-hospital stroke and mortality among women and men (11.6% [CAS] vs 2.2% [CEA]; P = .024).In the Washington state Vascular-Interventional Surgical Care and Outcomes Assessment Program registry, hospital composite outcome of stroke and mortality following carotid interventions from 2010 to 2015 were noted to be similar for women and men. The notable exception to this finding was observed in subcohort of women undergoing CAS for symptomatic carotid disease at age 80 years or older. These findings should be taken into account when risk stratifying patients for carotid interventions.

Vascular Ehlers-Danlos syndrome (vEDS) is a connective tissue disorder due to defective type III collagen production and is associated with arterial rupture, spontaneous intestinal perforation, and gravid uterine rupture. Spontaneous pneumothorax and/or hemothorax (P/HTX) also occurs in vEDS patients. The temporal relation of pulmonary manifestations to arterial and intestinal complications in vEDS has not been well described. This was investigated in a multi-institutional retrospective case series of vEDS patients with confirmatory testing for COL3A1 mutation between 2000 and 2012. Data abstracted included demographics, family histories, presentation, and management of associated complications. Ninety-six cases (39% males, mean age 38.6 ± 15.5 years, range 8-79) had confirmatory testing for vEDS. P/HTX was documented in 17 (17.7%) cases. Most P/HTX preceded the diagnosis of vEDS (81%). Diagnosis of vEDS was made after arterial or intestinal complications at a mean of 7 years (range 0-26) post the initial P/HTX. In conclusion, spontaneous P/HTX is an early manifestation of vEDS frequently preceding an arterial complication or intestinal perforation. Thus, a spontaneous P/HTX in a young patient should trigger a differential diagnosis that includes vEDS. This should lead to an investigation of other vEDS features and subsequent genetic testing if vEDS features are present.

Objective Sarcopenia, defined as a loss of muscle mass or poor muscle quality, is a syndrome associated with poor surgical outcomes. The prognostic value of sarcopenia in patients with thoracoabdominal aortic aneurysms (TAAAs) is unknown. The present study was designed to define sarcopenia in this patient population and assess its impact on survival among patients who had undergone operative and nonoperative management of TAAAs. Methods We retrospectively reviewed all patients with a diagnosis of a TAAA at an academic hospital between 2009 and 2017 who had been selected for operative and nonoperative management. Sarcopenia was identified by measuring the total muscle area on a single axial computed tomography image at the third lumbar vertebra. The muscle areas were normalized by patient height, and cutoff values for sarcopenia were established at the lowest tertile of the normalized total muscle area. Long-term patient survival was assessed using Kaplan-Meier and Cox regression models. Results A total of 295 patients were identified, of whom 199 had undergone operative management and 96 nonoperative management for TAAAs. The patients selected for nonoperative management were more likely to be women and to have chronic kidney disease, coronary artery disease, cerebrovascular disease, a higher modified frailty index, and a larger aortic diameter. The Kaplan-Meier analyses revealed significantly lower long-term survival for the patients with and without sarcopenia in the operative and nonoperative groups. In Cox regression analyses, sarcopenia was a significant predictor of shorter survival for both operative (hazard ratio, 0.96; 95% confidence interval, 0.94-0.99; P = .006) and nonoperative (hazard ratio, 0.95; 95% confidence interval, 0.90-1.00; P = .05) groups after adjusting for age, race, sex, maximum aortic diameter, modified frailty index, chronic kidney disease, and active smoking. Additionally, age was a significant predictor of shorter survival in the operative group, and smoking and aortic diameter were significant in the nonoperative group. Conclusions In our cohort of patients who had received operative and nonoperative management of TAAAs, the patients with sarcopenia had had significantly lower long-term survival, regardless of whether surgery had been performed. These data suggest that sarcopenia could be used as a predictor of survival for patients with TAAAs and might be useful for risk stratification and decision making in the management of TAAAs.

Objective The first annual Women's Vascular Summit highlighted sex- and gender-related knowledge gaps in vascular disease diagnosis and treatment. This finding suggests an opportunity for further research to improve care and outcomes in people who identify as women, specifically. The purpose of this study was to a large national dataset to identify all operations performed for abdominal aortic aneurysm (AAA), carotid artery stenosis (CAS), and peripheral arterial disease (PAD) in the United States, and to provide data on sex-related disparities in treatment. Methods All hospitalizations of adult patients (≥18 years old) diagnosed with AAA, CAS, or PAD who underwent vascular surgery from 2000 to 2016 were identified in the Healthcare Cost and Utilization Project National Inpatient Sample. Sex-stratified U.S. Census data and sex-specific population disease prevalence estimates from the National Institute of Health and Agency for Healthcare Research and Quality were used to calculate the number of U.S. adults with AAA, CAS, and PAD. Sex-stratified rates of surgery and incidence rate ratios were estimated using Poisson regression. Among those undergoing surgery, multivariable logistic regression was used to assess differences in endovascular vs open approach. Results Over 16 years, there were 1,021,684 hospitalizations for vascular surgery: 13% AAA (21% female, 79% male), 40% CAS (42% female, 58% male), and 47% PAD (42% female, 58% male). Females were older than males at time of surgery (median age, 71.3 years vs 69.7 years) and less likely to have private insurance (18% vs 23%); minimal differences were seen across race/ethnicity, comorbidities, and hospital characteristics. After accounting for disease prevalence, females were still 25% less likely to undergo surgery for AAA and 30% less likely to undergo surgery for PAD compared with males with the same disease. These results were consistent over time. After adjustment, females, compared with males, were less likely to receive an endovascular procedure compared with open for AAA or CAS, and more likely to receive one for PAD. Conclusions From 2000 to 2016 in the United States, females were less likely to undergo intervention for AAA and PAD than males. This finding is particularly significant for PAD, because the prevalence is the same for both sexes, indicating that females are likely undertreated for PAD. Additionally, females were less likely to undergo endovascular surgery for AAA and more likely to undergo endovascular surgery for PAD than males. These findings suggest that improvement in AAA and PAD identification and management in females may improve outcomes.

OBJECTIVE. Vascular Ehlers-Danlos syndrome (vEDS) is a rare disorder and one of 13 types of Ehlers-Danlos syndrome (EDS). The syndrome results in aortic and arterial aneurysms and dissections at a young age. Diagnosis is confirmed with molecular testing via skin biopsy or genetic testing for COL3A1 pathogenic variants. We describe a multi-institutional experience in the diagnosis of vEDS from 2000 to 2015. METHODS. This is a multi-institutional cross-sectional retrospective study of individuals with vEDS. The institutions were recruited through the Vascular Low Frequency Disease Consortium. Individuals were identified using the International Classification of Diseases (ICD)-9 and 10-CM codes for EDS (756.83 and Q79.6). Review of records was then performed to select individuals with vEDS. Data abstraction included demographics, family history, clinical features, major and minor diagnostic criteria, and molecular testing results. Individuals were classified into two cohorts and then compared: those with pathogenic COL3A1 variants and those diagnosed by clinical criteria alone without molecular confirmation. RESULTS. Eleven institutions identified 173 (35.3% male, 56.6% Caucasian) individuals with vEDS. Of those, 11 (9.8%) had non-pathogenic alterations in COL3A1 and were excluded from the analysis. Among the remaining individuals, 86 (47.7% male, 68% Caucasian, 48.8% positive family history) had pathogenic COL3A1 variants and 76 (19.7% male, 19.7% Caucasian, 43.4% positive family history) were diagnosed by clinical criteria alone without molecular confirmation. Compared to the cohort with pathogenic COL3A1 variants, the clinical diagnosis only cohort had a higher number of females (80.3% vs. 52.3%, P

Purpose: Smooth muscle dysfunction syndrome (SMDS) due to heterozygous ACTA2 arginine 179 alterations is characterized by patent ductus arteriosus, vasculopathy (aneurysm and occlusive lesions), pulmonary arterial hypertension, and other complications in smooth muscle–dependent organs. We sought to define the clinical history of SMDS to develop recommendations for evaluation and management. Methods: Medical records of 33 patients with SMDS (median age 12 years) were abstracted and analyzed. Results: All patients had congenital mydriasis and related pupillary abnormalities at birth and presented in infancy with a patent ductus arteriosus or aortopulmonary window. Patients had cerebrovascular disease characterized by small vessel disease (hyperintense periventricular white matter lesions; 95%), intracranial artery stenosis (77%), ischemic strokes (27%), and seizures (18%). Twelve (36%) patients had thoracic aortic aneurysm repair or dissection at median age of 14 years and aortic disease was fully penetrant by the age of 25 years. Three (9%) patients had axillary artery aneurysms complicated by thromboembolic episodes. Nine patients died between the ages of 0.5 and 32 years due to aortic, pulmonary, or stroke complications, or unknown causes. Conclusion: Based on these data, recommendations are provided for the surveillance and management of SMDS to help prevent early-onset life-threatening complications.

Women have been shown to experience inferior outcomes following intact and ruptured abdominal aortic aneurysm (AAA) treatment in endovascular aneurysm repair (EVAR) and open surgical repair (OSR) groups. The goal of our study was to compare gender-specific presentation, management, and early outcomes after AAA repair using a statewide registry.We utilized the Washington State's Vascular Interventional Surgical Care and Outcomes Assessment Program registry data collected in 19 hospitals from July 2010 to September 2013. Demographics, presentation, procedural data, and outcomes in elective and emergent AAA repair groups were analyzed.We identified 1,231 patients (19.6% women) who underwent intact (86.4%) or ruptured AAA (13.6%) repairs. Nine thousand seventy-two (79.0%) patients had EVAR and 259 (21.0%) had OSR. Men and women were of equivalent age and had similar comorbidities, except that women had less coronary artery disease (P 0.01) and were more likely to suffer from chronic obstructive pulmonary disease (P = 0.05). Women had smaller aneurysm diameters (5.8 ± 1.1 vs. 6.2 ± 1.8 cm, P 0.01) at the time of presentation and men had slightly higher incidence of rupture at larger aneurysm size. Men were more likely to undergo EVAR, with significant differences in elective (82.1% vs. 74.1%, P = 0.01), but not ruptured repair. Women had significantly higher mortality rates following elective EVAR (3.1% vs. 0.6%, P = 0.01), but not after ruptured or elective open repair. Following elective EVAR, women were less likely to be discharged to home after longer hospital stays (3 vs. 2 days, P 0.01).Despite presentation at a similar age, with a smaller aneurysm diameter, and similar medical comorbidities, women experience substantially worse hospital outcomes primarily driven by elective endovascular procedures. Utilization of endovascular techniques in women still remains lower compared with men. Improvement of elective outcomes in women will likely depend on technical advancements in repair techniques and management strategies that may differ between genders.

Objective Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder owing to pathogenic variants in COL3A1 that lead to impaired type III collagen production. We aim to describe the contemporary multi-institutional experience of aortic and arterial pathology in individuals with vEDS, to evaluate disease patterns and refine management recommendations. Methods This cross-sectional, retrospective study of individuals with genetically confirmed vEDS was conducted between 2000 and 2015 at multiple institutions participating in the Vascular Low Frequency Disease Consortium. Aortic and arterial events including aneurysms, pseudoaneurysms, dissections, fistulae, or ruptures were studied. Demographics, COL3A1 variants, management, and outcomes data were collected and analyzed. Individuals with and without arterial events were compared. Results Eleven institutions identified 86 individuals with pathogenic variants in COL3A1 (47.7% male, 86% Caucasian; median age, 41 years; interquartile range [IQR], 31.0-49.5 years; 65.1% missense COL3A1 variants). The median follow-up from the time of vEDS diagnosis was 7.5 years (IQR, 3.5-12.0 years). A total of 139 aortic/arterial pathologies were diagnosed in 53 individuals (61.6%; 50.9% male; 88.5% Caucasian; median age, 33 years; IQR, 25.0-42.3 years). The aortic/arterial events presented as an emergency in 52 cases (37.4%). The most commonly affected arteries were the mesenteric arteries (31.7%), followed by cerebrovascular (16.5%), iliac (16.5%), and renal arteries (12.2%). The most common management was medical management. When undertaken, the predominant endovascular interventions were arterial embolization of medium sized arteries (13.4%), followed by stenting (2.5%). Aortic pathology was noted in 17 individuals (32%; 58.8% male; 94.1% Caucasian; median age, 38.5 years; IQR, 30.8-44.7 years). Most notably, four individuals underwent successful abdominal aortic aneurysm repair with excellent results on follow-up. Individuals with missense mutations, in which glycine was substituted with a large amino acid, had an earlier onset of aortic/arterial pathology (median age, 30 years; IQR, 23.5-37 years) compared with the other pathogenic COL3A1 variants (median age, 36 years; IQR, 29.5-44.8 years; P = .065). There were 12 deaths (22.6%) at a median age of 36 years (IQR, 28-51 years). Conclusions Most of the vEDS arterial manifestations were managed medically in this cohort. When intervention is required for an enlarging aneurysm or rupture, embolization, and less frequently stenting, seem to be well-tolerated. Open repair of abdominal aortic aneurysm seems to be as well-tolerated as in those without vEDS; vEDS should not be a deterrent to offering an operation. Future work to elucidate the role of surgical interventions and refine management recommendations in the context of patient centered outcomes is warranted.

Data regarding the cardiac abnormalities associated with Stanford type B aortic dissection (TBAD) and whether these abnormalities are related to outcomes are limited. We describe the prevalence of cardiac abnormalities in patients with TBAD as detected by echocardiography.This retrospective review included patients with TBAD presenting between 1990 and 2016. Echocardiograms performed within 6 weeks of acute TBAD were reviewed. Cardiac function, valve abnormalities, and stigmata of hypertensive heart disease including left ventricular hypertrophy (LVH) were ascertained. Characteristics of patients who did and did not receive echocardiograms were compared. Outcomes of patients with and without evidence of LVH on echocardiography were also compared.Of 239 patients with TBAD, 90 had echocardiograms performed within 6 weeks of acute TBAD (74% male; mean age, 57.8 ± 13.2 years). Echocardiograms were obtained at a median of 2 days (range, 0-41 days) from acute TBAD. Patients who had echocardiograms were more likely to present with malperfusion (28% vs 14%; P .01) and had a trend toward increased operative repair during the subacute phase (17.4% vs 9.5%; P = .07) compared with patients who did not receive an echocardiogram. A majority of patients (57%) had at least mild LVH, including 39% of patients without a prior diagnosis of hypertension. Fibrocalcific changes associated with hypertension, including aortic sclerosis and mitral annular calcification, were noted in 40% and 11% of the patients, respectively. Among patients with LVH, there was a trend toward higher all-cause mortality (35% vs 23%; P = .21) and a younger age at death (58 ± 14 years vs 66 ± 13 years; P = .19) despite a similar age at TBAD onset. In a multivariable analysis controlling for age, sex, and admission estimated glomerular filtration rate, LVH independently predicted all-cause mortality (hazard ratio, 2.38; 95% confidence interval, 1.02-5.56; P = .04).LVH and other findings of hypertensive heart disease are common in patients with TBAD. LVH predicted all-cause mortality after TBAD in this small group of patients. Further exploration of the relationship between the chronic effects of hypertension and using LVH as an objective biomarker to risk stratify patients with TBAD and long-term outcomes after TBAD is warranted.

Background The coronavirus disease 2019 (COVID-19) pandemic has led to unprecedented challenges for health care systems globally. We designed and administered a global survey to examine the effects of COVID-19 on vascular surgeons and explore the COVID-19-related stressors faced, coping strategies used, and support structures available. Methods The Pandemic Practice, Anxiety, Coping, and Support Survey for Vascular Surgeons was an anonymous cross-sectional survey sponsored by the Society for Vascular Surgery Wellness Task Force. The survey analysis evaluated the effects of COVID-19-related stressors on vascular surgeons measured using the Generalized Anxiety Disorder 7-item scale. The 28-item Brief Coping Orientation to Problems Experienced inventory was used to assess the active and avoidant coping strategies. Survey data were collected using REDCap (Research Electronic Data Capture) from April 14, 2020 to April 24, 2020 inclusive. Additional qualitative data were collected using open-ended questions. Univariable and multivariable analyses of the factors associated with the anxiety levels and qualitative analysis were performed. Results A total of 1609 survey responses (70.5% male; 82.5% vascular surgeons in practice) from 58 countries (43.4% from United States; 43.4% from Brazil) were eligible for analysis. Some degree of anxiety was reported by 54.5% of the respondents, and 23.3% reported moderate or severe anxiety. Most respondents (∼60%) reported using active coping strategies and the avoidant coping strategy of “self-distraction,” and 20% used other avoidant coping strategies. Multivariable analysis identified the following factors as significantly associated with increased self-reported anxiety levels: staying in a separate room at home or staying at the hospital or a hotel after work (odds ratio [OR], 1.39; 95% confidence interval [CI], 1.08-1.79), donning and doffing personal protective equipment (OR, 1.81; 95% CI, 1.41-2.33), worry about potential adverse patient outcomes due to care delay (OR, 1.47; 95% CI, 1.16-1.87), and financial concerns (OR, 1.90; 95% CI, 1.49-2.42). The factors significantly associated with decreased self-reported anxiety levels were hospital support (OR, 0.83; 95% CI, 0.76-0.91) and the use of positive reframing as an active coping strategy (OR, 0.88; 95% CI , 0.81-0.95). Conclusions Vascular surgeons globally have been experiencing multiple COVID-19-related stressors during this devastating crisis. These findings have highlighted the continued need for hospital systems to support their vascular surgeons and the importance of national societies to continue to invest in peer-support programs as paramount to promoting the well-being of vascular surgeons during and after the COVID-19 pandemic.

Acute aortic dissections are a preventable cause of sudden death if individuals at risk are identified and surgically repaired in a non-emergency setting. Although mutations in single genes can be used to identify at-risk individuals, the majority of dissection case subjects do not have evidence of a single gene disorder, but rather have the other major risk factor for dissections, hypertension. Initial genome-wide association studies (GWASs) identified SNPs at the FBN1 locus associated with both thoracic aortic aneurysms and dissections. Here, we used the Illumina HumanExome array to genotype 753 individuals of European descent presenting specifically with non-familial, sporadic thoracic aortic dissection (STAD) and compared them to the genotypes of 2,259 control subjects from the Atherosclerosis Risk in Communities (ARIC) study matched for age, gender, and, for the majority of cases, hypertension. SNPs in FBN1, LRP1, and ULK4 were identified to be significantly associated with STAD, and these results were replicated in two independent cohorts. Combining the data from all cohorts confirmed an inverse association between LRP1 rs11172113 and STAD (p = 2.74 × 10(-8); OR = 0.82, 95% CI = 0.76-0.89) and a direct association between ULK4 rs2272007 and STAD (p = 1.15 × 10(-9); OR = 1.35, 95% CI = 1.23-1.49). Genomic copy-number variation analysis independently confirmed that ULK4 deletions were significantly associated with development of thoracic aortic disease. These results indicate that genetic variations in LRP1 and ULK4 contribute to risk for presenting with an acute aortic dissection.

While a genetic basis for thoracic aortic aneurysm and dissection is well established, little is known about endovascular stent graft therapy (TEVAR) outcomes in patients with family history of aortic disease following Type B acute aortic dissection (TBAD). Patients enrolled in the International

Vertebral artery tortuosity has been associated with adverse events in Marfan and Loeys-Dietz syndromes, but has not been assessed in Vascular Ehlers-Danlos syndrome (VEDS). Subjects

BACKGROUND: Repair of thoracoabdominal aortic aneurysm (TAAA) is high risk and resource intensive prophylactic operation. A benefit from the operation is realized only by those patients who both survive the procedure and remain free from permanently disabling complications. The majority of literature describing treatment for TAAA consist of operative series reported by national centers of excellence. These studies are limited by referral and selection bias, and they exclude patients not suited for the reported modality of repair. Little is known about the characteristics and survival of those patients who are not referred or who are turned down for repair. For those undergoing an intervention, outcomes such as functional status after recovery from surgery are rarely reported. This study attempts to address these gaps by reporting both survival and a patient-centered “good” outcome in an inclusive cohort of patients with TAAA, including all nonoperative and operative patients irrespective of treatment modality. METHODS: A single institution database was screened by diagnosis codes for TAAA from 2009–2017 using the International Classification of Diseases (ICD) versions 9 and 10. Diagnosis was confirmed by retrospective chart review and the CT finding of aneurysmal degeneration > 3.2 cm of the paravisceral aorta in continuity with aneurysmal aorta meeting standard criteria for repair. Patients under age 18 and those with mycotic aneurysm were excluded. Patients were either managed nonoperatively or by means of one of four categories of repair: (1) open, (2) endovascular with branched endografts, (3) hybrid, defined as ilio-visceral debranching followed by endovascular repair with standard devices, and (4) partial repair in which the paravisceral segment was intentionally left unaddressed. The primary outcomes were (1) survival and (2) a composite “good” outcome defined as successful aneurysm exclusion, freedom from permanent loss of organ system function, and return to preoperative functional status after recovery from surgery (6–18 months). RESULTS: After review of CT imaging, 432 of 718 patients initially identified by ICD codes met inclusion criteria. Advanced medical comorbidities were seen in 33% of the entire cohort. Nonoperative management was utilized for 48% of the cohort with a 1-year survival of 65%. A survival benefit was seen in the open, endovascular and partial but not hybrid operative groups over the nonoperative group over a three-year period. Overall 1-year survival was 81%, but only 68% had a “good” outcome (p=0.0016). CONCLUSIONS: Close to half of the patients in this study did not undergo repair despite access to a variety of operative techniques, and many likely died in the short-term due to nonaneurysm-related causes. Among those who did undergo an operation, survival alone did not accurately represent the outcomes of operative intervention given the wide difference between survival and good outcome even with minimally invasive techniques. Operation appears to confer a survival advantage among appropriately selected patients, but a large proportion of patients with TAAA are unlikely to benefit from operative repair due to limited baseline survival and low probability of good outcome.

Objective The c.530G>A (p.Arg177Gln) mutation in PRKG1 has been shown to be associated with thoracic aortic aneurysms and dissections. This rare mutation accounts for an estimated 1% of nonsyndromic heritable thoracic aortic disease. We sought to describe the clinical presentation of type B aortic dissection (TBAD), management, and outcomes in patients with this mutation. Methods This is a descriptive multi-institutional retrospective study of patients from six families with the PRKG1 mutation. Patients with TBAD were selected for analysis. Demographics, family histories, TBAD management, and outcomes were reviewed. Results Of the 29 individuals diagnosed with the PRKG1 mutation, 12 (41.3%) had TBAD (50% male, TBAD median age: 31 years [range, 16-58 years], median follow-up: 6 years [range, 3-15 years] after TBAD). All had a family history of aortic dissections and none had features of Marfan syndrome. The median size of the descending thoracic aorta (DTA) at TBAD was 4.1 cm (range, 3.8-5 cm). Most cases (9 acute TBAD, 1 incidental TBAD diagnosis during screening) were managed medically. One case had open DTA repair the acute phase. Repair for dissection-related aneurysmal degeneration was performed in seven cases (58.3%) in the chronic phase at a median of 2 years (range, 1-8 years) after TBAD. In four cases (33.3%), the DTA remained stable in size over a range of 1 to 7 years after TBAD. Type A aortic dissection subsequent to TBAD occurred in three cases (25%). There were four (33.3%) deaths in the series, all aortic related at a median age of 24 years (range, 19-43 years). Conclusions The PRKG1 (p.Arg177Gln) mutation although rare is associated with nonsyndromic TBAD in young and middle-aged patients. Workup for this gene mutation should be included as part of the workup for TBAD etiology in relatively young patients and those with familial history of aortic dissections. Once diagnosed, testing of first-degree family members is warranted. In all individuals with a PRKG1 mutation, close follow-up for aortic root dilatation and hypertension control is essential to reduce the risk of type A or type B aortic dissection, and in cases of TBAD, to decrease the risk of dissection-related aneurysmal degeneration.

The clinical application of serum biomarkers (d-dimer, C-reactive protein) to predict the natural history of descending thoracic aortic dissection remains elusive. In this review, our current understanding of biomarkers in descending thoracic aortic dissection detection, predicting complications, and aiding in patient management is discussed.

Congenital aortic arch variations are more common in patients with thoracic aortic disease for reasons unknown. Additionally, little is understood about their relation to type B aortic dissections (TBAD) specifically. We investigated the prevalence of variant aortic arch anatomy in patients with TBAD compared with controls. To understand the implications of how variant aortic arch anatomy may contribute to degenerative aortic disease, we compared flow hemodynamics of three variations of aortic arches using four-dimensional flow magnetic resonance imaging (4D flow MRI).Arch anatomy on computed tomography imaging was reviewed and compared between patients with TBAD and age/sex-matched controls free of aortic pathology. Arch variants were defined as follows: common origin of innominate and left common carotid artery (bovine arch), aberrant right subclavian artery, and right-sided aortic arch. Demographics, TBAD characteristics, and follow-up data were abstracted. Patients with TBAD with variant and conventional aortic arches were compared. Additionally, three matched healthy controls with conventional, bovine, and aberrant right subclavian artery arches underwent 4D flow MRI evaluation to assess if there were differences in flow patterns by arch type. Indices of regional hemodynamic wall sheer stress were compared.Computed tomography scans of 185 patients with TBAD (mean age, 58.1 ± 12.4 years; 72.4% males; 71.4% Caucasian) and 367 controls (mean age, 62.5 ± 13.4 years; 67% males; 77.9% Caucasian) were reviewed. Variant arch anatomy was more prevalent in patients with TBAD (40.5% vs 24.5%; P .001). In patients with TBAD, there were no differences in the mean age of presentation and descending thoracic aorta diameter among those with variant or conventional arch anatomy. Patients with TBAD with variant arch anatomy had a higher percentage of dissection related thoracic aortic repairs (54.7% vs 33.6%; P = .004) with repairs occurring predominantly in the acute phase. 4D flow MRI demonstrated a higher systolic wall shear stress along the inner curve of the bovine arch compared with the conventional aberrant right subclavian artery arches.Variant aortic arch anatomy is significantly more prevalent in patients with TBAD. patients with TBAD with variant arch anatomy had a higher percentage of dissection-related aortic repair. Preliminary 4D flow MRI data show differences in hemodynamic flow patterns between variant and conventional arches. Studies of long-term outcomes based on arch anatomy may offer additional insight to TBAD genesis and possibly influence management decisions.

Objective Endovascular repair in patients with connective tissues disorders is not recommended because of concern for repair failure. The aim of this study was to investigate thoracic endovascular aortic repair (TEVAR) outcomes in patients with confirmed or suspected syndromic and nonsyndromic genetically triggered thoracic aortic dissection. Methods We analyzed data for patients with descending thoracic aorta (DTA) dissection treated with TEVAR from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). Enrolled patients had confirmed (syndromic or familial) or suspected genetically triggered thoracic aortic disease. The latter group includes patients with sporadic aortic dissection presenting at 50 years of age or younger in the absence of a family history or syndromic features. Results Between 2006 and 2014, there were 371 patients with DTA dissection enrolled in GenTAC. TEVAR was performed in 31 cases (58.1% male; median age, 47 years; range, 21.3-65.6 years). Genetically triggered aortic dissection was confirmed in 18 cases, and an additional 13 cases had suspected genetically triggered dissection because of early onset of presentation. TEVAR was performed in nine patients with type A aortic dissection: five in conjunction with acute type A dissection repair and four in the chronic phase to treat aneurysmal degeneration of the residual dissected DTA (median interval to TEVAR, 2.1 years). TEVAR was also performed in 22 cases of type B aortic dissection (TBAD), 12 acute and 10 chronic (median interval to TEVAR, 1.6 years). There were no perioperative deaths. Median follow-up for all cases was 2 years (range, 0.4 month-7 years). Reinterventions after TEVAR were performed in 13 cases (41.9%). This included urgent repair of three retrograde ascending aorta dissections occurring after TEVAR for acute TBAD (25%) and seven thoracoabdominal repairs with stent graft explantation (22.6%) at a median of 7 months after TEVAR (range, 1-16.6 months). Conclusions TEVAR in patients with genetically triggered aortic dissections can be lifesaving in the acute setting though associated with high risk of retrograde aortic dissection in acute TBAD. For chronic dissection-related DTA aneurysmal degeneration, TEVAR could potentially be lifesaving in patients deemed too high risk for open surgical repair. Close postoperative surveillance is required, given the risk of subsequent device failure and need for reintervention. Because these circumstances are rare, multicenter prospective enrollment of patients with genetically triggered aortic disease is essential to delineate the indications for and risks of TEVAR in this heterogeneous population.

Objective The contemporary practice of testing for genetically triggered aortic and arterial aneurysms and dissections is not well described. This study aimed to describe this practice at a tertiary care academic center and to ascertain the yield of testing in establishing the diagnosis in patients referred on the basis of clinical suspicion. Methods This is a retrospective cohort study of patients referred for vascular genetic testing at an academic medical center between 2010 and 2015. Patients were identified by Current Procedural Terminology diagnostic codes 81405, 81408, and 81479 for genetic testing (Marfan syndrome, Loeys-Dietz syndrome, aneurysms-osteoarthritis syndrome, COL3A1 , and familial thoracic aortic aneurysm panel [ ACTA2 , COL3A1 , TGFBR1 , TGFBR2 , SMAD3 , TGFB2 , MYLK , MYH11 , and PRKG1 genes]) and by review of the collagen vascular laboratory database for genetic testing results. Data abstracted included demographics, clinical history, reason for referral, family history, referring provider type, and outcomes of genetic testing. Results Ninety-six patients (44.3% male; median age, 40.8 years) were referred for suspected genetic vascular disease. Genetic testing was performed in 75 cases thought to have heritable mutations related to aortic or arterial aneurysms and dissections. The most common reason for genetic testing was a personal history of aortic or arterial aneurysms and dissections (62.3%; mean age, 45.8 ± 11.1 years), followed by a family history of aortic or arterial aneurysms and dissections without a personal history (26.6%; age, 28.8 ± 17.9 years). The most common genetic testing performed was a familial thoracic aortic aneurysm gene panel (44%), followed by single gene testing for vascular Ehlers-Danlos syndrome (33.3%). Genetic testing identified a pathogenic mutation in 36% of the cases. The highest likelihood of identifying a pathogenic mutation was in those who had a family history with an already diagnosed mutation (57.1%), followed by patients with aortic root and ascending aortic aneurysm or dissection (42.3%). Conclusions In patients with suspected genetically triggered vascular disease, the yield of clinical vascular genetic testing is reasonable when selective genetic testing is performed on the basis of personal or family history. These tests should be obtained with appropriate expertise in genetic counseling and interpretation of genetic testing results. Negative genetic test results in the setting of a positive family history demonstrate the limits of testing and known mutations leading to genetically triggered aortic and arterial aneurysms and dissections and support the need for novel gene discovery.

The development of aortic endovascular repair has been a major advance in the treatment of aortic aneurysms and dissections. The use of endovascular repairs in patients with connective tissue disease has been limited due to concerns for device failure and they have been intentionally excluded from FDA trials. These patients form a subset of a larger group of patients with genetically triggered aortic disease. The aim of this chapter is to summarize the current body of knowledge about the genetics of syndromic (including connective tissue disease) and non-syndromic genetically triggered aortic aneurysms and dissections, diagnosis, current management, and role of endovascular repair in this population. Having a working knowledge of the known mutations leading to aortic aneurysms and dissections will allow the vascular surgeon to identify these patients and will facilitate early diagnosis and treatments tailored to the affected individuals.

BackgroundAdventitial cystic disease (ACD) is an unusual arteriopathy; case reports and small series constitute the available literature regarding treatment. We sought to examine the presentation, contemporary management, and long-term outcomes using a multi-institutional database.MethodsUsing a standardized database, 14 institutions retrospectively collected demographics, comorbidities, presentation/symptoms, imaging, treatment, and follow-up data on consecutive patients treated for ACD during a 10-year period, using Society for Vascular Surgery reporting standards for limb ischemia. Univariate and multivariate analyses were performed comparing treatment methods and factors associated with recurrent intervention. Life-table analysis was performed to estimate the freedom from reintervention in comparing the various treatment modalities.ResultsForty-seven patients (32 men, 15 women; mean age, 43 years) were identified with ACD involving the popliteal artery (n = 41), radial artery (n = 3), superficial/common femoral artery (n = 2), and common femoral vein (n = 1). Lower extremity claudication was seen in 93% of ACD of the leg arteries, whereas patients with upper extremity ACD had hand or arm pain. Preoperative diagnosis was made in 88% of patients, primarily using cross-sectional imaging of the lower extremity; mean lower extremity ankle-brachial index was 0.71 in the affected limb. Forty-one patients with lower extremity ACD underwent operative repair (resection with interposition graft, 21 patients; cyst resection, 13 patients; cyst resection with bypass graft, 5 patients; cyst resection with patch, 2 patients). Two patients with upper extremity ACD underwent cyst drainage without resection or arterial reconstruction. Complications, including graft infection, thrombosis, hematoma, and wound dehiscence, occurred in 12% of patients. Mean lower extremity ankle-brachial index at 3 months postoperatively improved to 1.07 (P < .001), with an overall mean follow-up of 20 months (range, 0.33-9 years). Eight patients (18%) with lower extremity arterial ACD required reintervention (redo cyst resection, one; thrombectomy, three; redo bypass, one; balloon angioplasty, three) after a mean of 70 days with symptom relief in 88%. Lower extremity patients who underwent cyst resection and interposition or bypass graft were less likely to require reintervention (P = .04). One patient with lower extremity ACD required an above-knee amputation for extensive tissue loss.ConclusionsThis multi-institutional, contemporary experience of ACD examines the treatment and outcomes of ACD. The majority of patients can be identified preoperatively; surgical repair, consisting of cyst excision with arterial reconstruction or bypass alone, provides the best long-term symptomatic relief and reduced need for intervention to maintain patency.

ObjectiveThe management of arterial pathology in individuals with vascular Ehlers-Danlos syndrome (vEDS) remains a challenge. Here we describe the correlation between COL3A1 gene mutation type and the clinical phenotype in individuals with vEDS.MethodsIndividuals with confirmed molecular diagnoses of vEDS were enrolled in a multi-institutional natural history study. Data collected included demographics, clinical and family histories, arterial pathology (aneurysm, dissection, and rupture), operative details, and autopsy reports. Individuals were classified into two cohorts by the type of COL3A1 mutations and their effect on the amount of normal collagen produced: those with mutations that lead to minimal (MIN) production (10%-15%) of normal type III collagen and those with haploinsufficiency (HI) mutations that lead to production of 50% of the normal type III collagen.ResultsA cohort of 68 individuals (72%) from 56 families had arterial pathology (44% male) with 13% HI. The HI group was older at the time of their first vascular event (mean, 42 [range, 26-58] years vs 33 [range, 8-62] years; P = .016) and had a higher incidence of aortic pathology than the MIN group (56% vs 21%; P = .025). Visceral arterial pathology was seen in 43 arteries in 23 individuals in the MIN group vs only one artery in five individuals in the HI group. Emergency surgical procedures were more likely to be undertaken when vEDS diagnosis was not known (81% vs 41%; P = .005), and 81% of these procedures were open surgical repair compared with 19% endovascular repairs (P = .019). Open and endovascular repairs were equally used in the elective setting. Postoperative complications were highest when the diagnosis of vEDS was not known (62% vs 14%; P < .001) and when procedures were undertaken in an emergency setting (5% vs 55% P < .001). Mortality due to arterial complications was 0% in the HI cohort and 21% in the MIN cohort (P = .132).ConclusionsArterial pathology in vEDS individuals is related to the underlying COL3A1 mutation type. The arterial pathology in individuals with HI mutations occurs at later ages with a higher incidence of aortic disease compared with other COL3A1 mutation types. Molecular diagnosis is recommended because diagnosis confirmation, appropriate surveillance, and prophylactic interventions in an elective setting improve surgical outcomes.

A new murmur of aortic insufficiency is a classic physical examination sign of acute aortic dissection (AAD); we sought to further describe AAD patients presenting with aortic insufficiency murmur without a prior history of aortic valve disease or repair. Patients enrolled from January 1996- June

Aneurysms of the internal mammary artery are rare. We describe a case of a 49-year-old woman with a SMAD3 mutation who presented with left internal mammary artery aneurysm that was thought to have ruptured, causing a large spontaneous left mediastinal hematoma. The aneurysm was treated successfully months after initial presentation with coil embolization. SMAD3 mutations are linked to familial thoracic aortic aneurysms and dissections, peripheral aneurysms, and early-onset osteoarthritis, with an estimated incidence of 2% in families with familial thoracic aortic aneurysms and dissections. To our knowledge, this is the first case in the literature to link a SMAD3 mutation with internal mammary artery aneurysm.

Isolated dissection of the mesenteric vessels is rare but increasingly recognized. This study aimed to evaluate patient characteristics, primary treatment, and subsequent outcomes of mesenteric dissection using multi-institutional data.All patients at participant hospitals between January 2003 and December 2015 with dissection of the celiac artery (or its branches) or dissection of the superior mesenteric artery (SMA) were included. Patients with an aortic dissection were excluded. Demographic, treatment, and follow-up data were collected. The primary outcomes included late vessel thrombosis (LVT) and aneurysmal degeneration (AD).Twelve institutions identified 227 patients (220 with complete treatment records) with a mean age of 55 ± 12.5 years. Median time to last follow up was 15 months (interquartile range, 3.8-32). Most patients were men (82% vs 18% women) and symptomatic at presentation (162 vs 65 asymptomatic). Isolated SMA dissection was more common than celiac artery dissection (n = 158 and 81, respectively). Concomitant dissection of both arteries was rare (n = 12). The mean dissection length was significantly longer in symptomatic patients than in asymptomatic patients in both the celiac artery (27 vs 18 mm; P = .01) and the SMA (64 vs 40 mm; P .001). Primary treatment was medical in 146 patients with oral anticoagulation or antiplatelet therapy (n = 76 and 70, respectively), whereas 56 patients were observed. LVT occurred in six patients, and 16 patients developed AD (3% and 8%, respectively). For symptomatic patients without evidence of ischemia (n = 134), there was no difference in occurrence of LVT with medical therapy compared with observation alone (9% vs 0%; P = .35). No asymptomatic patient (n = 64) had an episode of LVT at 5 years. AD rates did not differ among symptomatic patients without ischemia treated with medical therapy or observed (9% vs 5%; P = .95). Surgical or endovascular intervention was performed in 18 patients (3 ischemia, 13 pain, 1 AD, 1 asymptomatic). Excluding the patients treated for ischemia, there was no difference in LVT with surgical intervention vs medical management (one vs five; P = .57).Asymptomatic patients with isolated mesenteric artery dissection may be observed and followed up with intermittent imaging. Symptomatic patients tend to have longer dissections than asymptomatic patients. Symptomatic isolated mesenteric artery dissection without evidence of ischemia does not require anticoagulation and may be treated with antiplatelet therapy or observation alone.

A true aneurysm contains all layers of a venous wall (the intima, media, and adventitia) as compared to a pseudoaneurysm which is lined by fibrous tissue and thrombus. Aneurysmal degeneration of an arteriovenous fistula (AVF) is one of the most common complications of long-term AVF. This can be asymptomatic but can also lead to a broad spectrum of complications. The focus of this chapter is the discussion of aneurysmal degeneration in arteriovenous fistulas.

A proper physical examination is an invaluable tool in discovering emerging problems of a vascular access in a timely fashion, thus preventing serious complications, potential loss, and poor outcomes. Though subjective in nature, with experience this can be performed quickly and accurately and is considered a critical part of patient care. The 2006 National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) and the 2008 Society for Vascular Surgery practice guidelines recommend that physical examination be performed on all mature arteriovenous fistulas (AVFs) on a weekly basis. Like any physical examination the three elements include inspection, palpation, and auscultation. Basic knowledge of blood flow dynamics as discussed in the previous chapter is helpful in understanding the steps of physical examination and evaluation of findings. This chapter focuses on the physical examination of the newly created and the mature arteriovenous fistulas (AVF) and grafts (AVG).

True aneurysms of the internal mammary artery are rare and have been described in association with vasculitis or connective tissue disorders. Herein, we describe 2 cases of familial internal mammary artery aneurysms (IMAs) in 2 sisters with SMAD3 mutation. The older sister presented at the age of 54 years with an incidental diagnosis of a multilobed right IMA and the younger sister presented several years earlier with a ruptured left IMA aneurysm at the age of 49 years. Both sisters had Debakey type I aortic dissections prior to the IMA aneurysm presentation. To our knowledge, this is the first time IMA aneurysms have been described in siblings with SMAD3 mutation. In our experience, endovascular repair is a feasible and safe treatment option. An assessment of the entire arterial tree is recommended in patients diagnosed with SMAD3 mutations.

Once seen as an exotic option, endovascular techniques for stopping hemorrhage and for restoring perfusion in patients with vascular trauma are increasingly commonplace in the specialist setting. The principle methods are catheter-directed coil embolization and stent-graft deployment; either may be used in the acute phase or to treat established fistulae and pseudoaneurysms. The principle benefits relate to avoidance of excessive morbidity that may accompany the extensive dissection required to control difficult-to-reach vascular structures. Stent grafts may be used as damage control or as definitive measures, with injuries to the carotid artery, the axillosubclavian axis, and the thoracic aorta most amenable to this technique. In particular, endovascular stent-graft repair appears to have a much better short- and medium-term safety profile compared to traditional open repair for blunt injury of the thoracic aorta, although the life-time durability of stents remains to be seen. In patients with severe hemorrhagic shock, endovascular deployment of an aortic occlusive balloon avoids the requirement for thoracotomy and aortic cross-clamp. Areas for development in endovascular therapy for trauma include improved stent design and durability suited to a younger population, better characterization of the indications for endovascular intervention in different categories of vessel trauma, the place and duration of antiplatelet therapy, and improved follow-up of stent recipients.

Background Blunt abdominal aortic injury (BAAI) is a rare injury with less than 200 cases in the current reported world literature, mostly in case report format. We sought to describe the experience of a high-volume trauma center and to provide a contemporary review of the literature to better understand the natural history and management of this injury. Methods This was a retrospective review of patients with BAAI between 1996 and 2010. Data collected included demographics, mechanism of injury, associated injuries, type of intervention, subsequent imaging, and follow-up. BAAI was classified by the presence of external aortic contour abnormality noted as an intimal tear, large intimal flap, pseudoaneurysm, or free rupture. Abdominal aorta zones of injury were classified by possible surgical approaches as zone I (diaphragmatic hiatus to superior mesenteric artery [SMA]), zone II (includes SMA and renal arteries), and zone III (from the inferior aspect of the renal arteries to the aortic bifurcation). Results We identified 28 individuals (68% male) with BAAI (median age, 28.5; range, 6-61 years). The median injury severity score was 45 (range, 16-75), and 39% were hypotensive at presentation. BAAI presented as intimal tear (21%), large intimal flap (39%), pseudoaneurysm (11%), and free rupture (29%). Zone III was the most common location of injury. Management depended on the location and type of injury: nonoperative (32%), open aortic repair (36%), endovascular repair (21%), and multimodality (10%). Overall mortality was 32%. Most deaths occurred during the initial operative exploration. The mortality rate of free aortic rupture was 100%. Intimal tears resolved or remained stable. Median follow-up was 15.5 months (range, 8 days-7.5 years). Vascular complications due to repair included a thrombosed access femoral artery during an endovascular repair and death of a patient who underwent a hybrid repair. Conclusions This is the largest BAAI series described in the English literature at one institution. BAAIs range from intimal tears to free rupture, with outcomes and management correlating with type and location of injury. Nonoperative management with blood pressure control using β-blockers coupled with antiplatelet therapy and close follow-up is successful in individuals with intimal tears with minimal thrombus formation because they remain stable or resolve on follow-up. Free rupture remains a devastating injury, with 100% mortality. For all other categories of aortic injury, successful repair correlates with a favorable prognosis.

Background: Pneumomediastinum occurs in up to 10% of patients with blunt thoracic and cervical trauma. Mandatory evaluation of all patients with bronchoscopy and esophageal imaging to exclude a major injury has been recommended. There is little data on the safety or efficacy of this approach. We evaluated the incidence of major injuries associated with pneumomediastinum, the accuracy of diagnostic modalities, and the results of observation versus aggressive evaluation. Methods: Medical records of all blunt trauma patients diagnosed with pneumomediastinum and/or aerodigestive tract injury between 1998 and 2005 were reviewed. The patient's hospital course was reviewed for demographic data, admission diagnoses, diagnostic imaging and procedures, operations, missed injuries, length of stay, and mortality. Results: The review identified a total of 136 patients with pneumomediastinum, and an additional 22 patients with thoracic aerodigestive tract injuries but without pneumomediastinum. Only patients with pneumomediastinum were considered in subsequent analysis. Pneumomediastinum was detected by CT scan in all 136 (100%) patients, although identified on plain radiograph in only 20 (15%) patients. Computed tomography findings were suspicious for a major aerodigestive tract injury in 27 (20%) patients. Ten (37%) of these 27 patients had an injury requiring operative intervention: five (4%) laryngeal injuries, three (2%) tracheal disruptions, and two (1%) esophageal perforations. Eighty-one patients (60%) never had endoscopic evaluation. There were no delayed diagnoses, missed injuries, or complications in the observation-only cohort. The overall sensitivity and specificity of CT scan for major aerodigestive tract injury was 100% and 85%, respectively. Conclusion: Major airway or esophageal injury is an uncommon cause of pneumomediastinum. CT scan was able to identify patients at high risk for aerodigestive injury in all cases, and should be the preferred screening tool for airway injury in patients with pneumomediastinum.

Background Damage-control laparotomy has become increasingly common after operative resuscitation of severe hemorrhagic shock after injury. Despite increased use, uncertainty exists about the safety and timing of enteral nutrition. The purpose of this study was to determine the safety and effect of immediate enteral nutrition. Study Design Data were obtained from a multicenter prospective cohort study evaluating clinical outcomes in adults with hemorrhagic shock after injury and were limited to patients with an open abdomen and no hollow viscus injury. The immediate enteral nutrition cohort was defined as initiation of enteral feeds within 36 hours after acute resuscitation completion. Multivariate stepwise logistic regression was used to evaluate factors associated with immediate enteral nutrition. Results One hundred subjects met inclusion criteria; 32 immediate enteral nutrition and 68 nonimmediate enteral nutrition. Nearly all patients underwent fascial closure (93.8% immediate enteral nutrition versus 94.1% nonimmediate enteral nutrition), with an average closure day of 6.47 ± 0.83 with immediate enteral nutrition and 8.55 ± 0.85 with nonimmediate enteral nutrition (p = 0.129). No significant difference in multiorgan dyfunction syndrome, length of ventilator days, ICU days, hospital days, or mortality was seen between groups. The rate of pneumonia was significantly different: 14 (43.8%) in immediate enteral nutrition and 49 (72.1%) in nonimmediate enteral nutrition (p = 0.008). Immediate enteral nutrition remained independently associated with a reduction in pneumonia within our stepwise regression (odds ratio=0.32; 95% CI, 0.13 to 0.79). Conclusions Immediate enteral nutrition after damage control appears safe, with no effect on abdominal closure rate. In addition, the reduction in pneumonia associated with immediate enteral nutrition suggests a tangible benefit. Immediate enteral nutrition should be considered in patients with open abdomens after severe trauma.

Paraesophageal hernia repair has been associated with a recurrence rate of up to 42%. Thus, in the last decade, there has been increasing interest in the use of mesh reinforcement of the hiatal repair. Polytetrafluoroethylene (PTFE) is one of the materials that have been used for this purpose, as it is thought to induce minimal tissue reaction. We report two cases in which complications specific to the use of PTFE mesh in this location developed over time. In the first patient, a gastrectomy was required to remove a large PTFE mesh which had eroded into the esophagogastric junction and gastric cardia. The second patient experienced severe dysphagia resulting from a stricture caused by the implant, requiring removal of the mesh. Although such complications have only rarely been reported, the severity and consequences of these incidents, as reported in the literature and in light of our observations, suggest that an alternative to PTFE should be considered for crural reinforcement during paraesophageal hernia repair.

Background: Thoracic Endovascular Aortic Repair (TEVAR) is often used to treat Type B acute aortic dissection. Although initially reserved for patients with complications, recent studies have also shown improved 5-year aorta-specific mortality in uncomplicated patients. Despite an increased focus on

Background: The genetic basis of thoracic aortic disease is well established. We sought to describe patients with Type B acute aortic dissection (TBAAD) with and without a family history (FH) of aortic disease and to compare the prevalence of FHbetween Type A and TBAAD. Methods: Patients enrolled

Objective Stent graft therapy has emerged as an alternative to open surgery in the management of chronic dissection-related aneurysmal degeneration (DRAD) in the descending thoracic aorta (DTA). The incidence of perioperative complications, need for secondary aortic intervention (SAI), and rate of aneurysmal false-lumen thrombosis have not been thoroughly described. Methods Perioperative and midterm outcomes in patients who underwent stent graft therapy for chronic DRAD DTA at a single institution between January 2006 and September 2013 were retrospectively analyzed. Preoperative anatomic factors, including the number of visceral and renal side branches off the false lumen, and false lumen volume, were analyzed for their ability to predict treatment failure. Treatment failure was defined as death, need for a SAI, and failure to achieve thrombosis of the DRAD DTA. Treatment success was defined as thrombosis of the false lumen in the area of the DRAD DTA with stability or a decrease in the maximum diameter of the DRAD DTA. Results During the study period, 47 patients underwent stent graft therapy for chronic DRAD DTA. Patients were a mean age of 58.3 ± 11.7 years, 74.5% (n = 35) were male, and 14.9% (n = 7) had a history of connective tissue disease. The left subclavian artery was covered in 48.9% (n = 23), and revascularization was performed in 87.0% (n = 20). Spinal drains were used in 74.5% (n = 35). Spinal cord ischemia developed in 6.4% (n = 3), which resolved in two and improved in one. No retrograde aortic dissections occurred. The 30-day mortality was 4.3% (n = 2); one death was in a patient with rupture. Mean clinical follow-up was 35.1 ± 20.9 months. The 5-year Kaplan-Meier survival was 89% ± 5%. Treatment failure occurred in 18 patients (38.3%): 9 required SAIs, 6 did not have thrombosis of the false lumen in the area of the DRAD DTA, and 4 died, with 1 patient dying during a SAI. No preoperative anatomic factor predicted treatment failure. The 5-year freedom from treatment failure was 54% ± 9%. Including the nine patients who underwent SAI, treatment success was achieved in 85.2% of patients. Conclusions In this single-center experience of stent graft therapy for chronic DRAD DTA, treatment success was achieved in 85% of patients after a SAI rate of 20%. No preoperative anatomic factor predicted treatment failure, which occurred in almost 40% of the patients. Identifying predictors of treatment failure may improve future outcomes.

BACKGROUND: Blunt abdominal aortic injury (BAAI) is a rare injury. The objective of the current study was to examine the presentation and management of BAAI at a multi-institutional level. METHODS: The Western Trauma Association Multi-Center Trials conducted a study of BAAI from 1996 to 2011. Data collected included demographics, injury mechanism, associated injuries, interventions, and complications. RESULTS: Of 392,315 blunt trauma patients, 113 (0.03%) presented with BAAI at 12 major trauma centers (67% male; median age, 38 years; range, 6-88; median Injury Severity Score [ISS], 34; range, 16-75). The leading cause of injury was motor vehicle collisions (60%). Hypotension was documented in 47% of the cases. The most commonly associated injuries were spine fractures (44%) and pneumothorax/hemothorax (42%). Solid organ, small bowel, and large bowel injuries occurred in 38%, 35%, and 28% respectively. BAAI presented as free aortic rupture (32%), pseudoaneurysm (16%), and injuries without aortic external contour abnormality on computed tomography such as large intimal flaps (34%) or intimal tears (18%). Open and endovascular repairs were undertaken as first-choice therapy in 43% and 15% of cases, respectively. Choice of management varied by type of BAAI: 89% of intimal tears were managed nonoperatively, and 96% of aortic ruptures were treated with open repair. Overall mortality was 39%, the majority (68%) occurring in the first 24 hours because of hemorrhage or cardiac arrest. The highest mortality was associated with Zone II aortic ruptures (92%). Follow-up was documented in 38% of live discharges. CONCLUSION: This is the largest BAAI series reported to date. BAAI presents as a spectrum of injury ranging from minimal aortic injury to aortic rupture. Nonoperative management is successful in uncomplicated cases without external aortic contour abnormality on computed tomography. Highest mortality occurred in free aortic ruptures, suggesting that alternative measures of early noncompressible torso hemorrhage control are warranted. LEVEL OF EVIDENCE: Multicenter retrospective review, level IV. Language: en

This study analyzed early and late outcomes after open repair of descending thoracic aortic aneurysms with chronic aortic dissection.We retrospectively reviewed our cases of open repair of descending thoracic aortic aneurysms with chronic dissection from 1991 to 2011. Patient comorbid conditions and operative details were analyzed to determine risks for adverse outcome. Long-term survival and aortic reinterventions were analyzed.We repaired 519 patients with descending thoracic aortic aneurysms during the study period, and 209 (40%) had chronic dissection. Mean age was 59 years, with 74% (154 of 209) men. Previous ascending repair was performed in 41% (85 of 209), and the second-stage elephant trunk was performed in 10% (21 of 209). Adjunctive distal aortic perfusion with cerebral spinal fluid drainage was used in 90% (188 of 209) of patients, and circulatory arrest with bypass in 1% (3 of 209). The 30-day mortality was 8.6% (18 of 209). Immediate neurologic deficit occurred in 0.95% (2 of 209) and only with extent C resection. Delayed neurologic deficit occurred in 1.4% (3 of 209), 1 patient in each extent. Because 66% (2 of 3) of the patients with delayed neurologic deficit recovered function, permanent deficit occurred in 1.4% (3 of 209). Stroke occurred in 2.4% (5 of 209) and dialysis on discharge in 5% (11 of 211). The only risk factor for 30-day mortality was preoperative glomerular filtration rate of less than 60 mL/min (odds ratio, 4.2; p0.006). Survival at 5, 10, and 15 years was 72%, 60%, and 49%, respectively. Freedom from reoperation on the operated-on segment was 98%, 96.5%, 96.5%, and 96.5% at 5, 10, 15, and 20 years.Open repairs of chronic descending thoracic aortic aneurysm dissections can be performed with respectable morbidity and mortality. Neurologic deficit remains low with the use of adjuncts, and early deaths are directly related to preoperative renal status. Reintervention on the involved aortic segment is low. These results allow comparisons with endovascular repair for chronic aortic dissection.

26 centers in theUnited States from2013 to2014.All patients were treated using the IBE, a bifurcated stent graft designed to be used with the existing Gore Excluder device for treatment of patients with CIA. The IBE device has limbs for extension into the external iliac and hypogastric artery to allow CIA exclusion with preservation of hypogastric artery blood flow. All patients underwent placement of a single IBE device, and patients with bilateral CIA were allowed to be enrolled with coil embolization and coverage of the contralateral internal iliac artery. Follow-up is ongoing and will include clinical examination and computed tomography imaging at 1 month, 6 months, 12 months, and yearly thereafter up to 5 years. Six-month site-reported data will be reported. Results: At the time of abstract submission, data were available for 53 patients (98% male). Median age was 68 years, and 35.2% of patients had unilateral CIA and 64.8% had bilateral CIA. Mean CIA diameter was 38.4 mm. Technical success was 92.5% (successful deployment and patency of all IBE components and freedom from type 1 or 3 endoleak). Median hospital stay was 1 day, and there was no 30-day mortality. Site-reported 30-day (n 1⁄4 48) and 6-month (n 1⁄4 19) follow-up data identified two IBE internal iliac limb occlusions, both asymptomatic, and one reintervention to treat an iliac artery dissection contralateral to the IBE device. Conclusions: Preliminary results of this prospective study demonstrate that treatment of CIA with the Gore IBE device is technically feasible and safe in the vast majority of appropriately selected patients. Initial results reveal high rates of patency of the internal iliac artery limb and freedom from new-onset erectile dysfunction or buttock claudication. Ongoing follow-up will seek to verify these results and to further assess durability.