Risk of pulmonary artery dissection in adults with congenital heart disease.

BACKGROUND: There are limited data about the risk of pulmonary artery (PA) dissection in adults with congenital heart disease (CHD), and the purpose of this study was to estimate the incidence of PA dissection in this population. METHODS: Retrospective cohort study of adults with CHD that underwent cross-sectional imaging (2003-2020). PA aneurysm was defined as main or branch PA diameter > 40 mm or > 30 mm respectively, and severe PA aneurysm was defined as main or branch PA diameter > 50 mm. RESULTS: Of 1, 673 patients (41 ± 10 years; male 58%), 493 (24%), 286 (19%), and 306 (20%) had aneurysms of the main, right, and left PA respectively, while 66 (4%) had severe PA aneurysm. During a median follow-up of 8.2 (interquartile range 3.7-10.3) years, there was one PA dissection in a patient with Eisenmenger syndrome, thus the incidence of PA dissection was 14 per 100,000 patient-years. Of 779 females, 163 had one or more pregnancies during follow-up, and 41 (25%) of these patients had known PA aneurysm at the time of conception. There was no PA dissection during pregnancy. Of the 163 patients, 91 (56%) had cross-sectional imaging before and after pregnancy, there was no significant difference in PA dimension before versus after pregnancy (main PA 35 ± 5 versus 36 ± 4 mm, p = 0.6; right PA 21 ± 3 versus 33 ± 4 mm, p = 0.1; and left PA 23 ± 4 versus 22 ± 4 mm, p = 0.4). CONCLUSIONS: The risk of PA dissection was extremely low even in patients with severe PA aneurysm, or in patients with PA aneurysm that became pregnant. Collectively, these data suggest a benign natural history for patients without severe pulmonary hypertension and encourage and a conservative approach in managing patients with PA aneurysm.

Natural history of renal artery aneurysms.

OBJECTIVE: The existing renal artery aneurysm (RAA) literature is largely composed of reports of patients who underwent intervention. The objective of this study was to review the natural history of RAA. METHODS: This single-institution retrospective analysis studied all patients with RAA diagnosed by computed tomography imaging between 2015 and 2019, identified by our institutional radiology database. Imaging, demographic, and clinical data were obtained via the electronic medical record. He growth rate was calculated for all patients with radiological follow-up. RESULTS: The cohort consists of 331 patients with 338 RAAs. Most patients were female (61.3%), with 11 (3.3%) of childbearing age. The mean age at diagnosis was 71.5 years (range, 24-99 years). Medical comorbidities included hypertension (73.7%), prior smoking (34.4%), and connective tissue disease (3.9%). Imaging indications included abdominal pain (33.5%), unrelated follow-up (29.6%), and follow-up of an RAA initially diagnosed before the study period (10.7%). Right RAA (61.9%) was more common than left (35.1%); 3% of patients had bilateral RAA. The mean diameter at diagnosis was 12.9 ± 5.9 mm. Size distribution included lesions measuring less than <15 mm (69.9%), 15 to 25 mm (27.1%), and more than 25 mm (3.0%). Anatomic locations included the distal RA (26.7%), renal hilum (42.4%), and mid-RA (13.1%). The majority were true aneurysms (98%); of these, 72.3% were fusiform and 27.7% were saccular. Additional characteristics included calcification (82.2%), thrombus (15.9%), and dissection (0.9%). Associated findings included aortic atherosclerosis (65.6%), additional visceral aneurysms (7.3%), and abdominal aortic aneurysm (5.7%). The mean clinical follow-up among 281 patients was 41.0 ± 24.0 months. The mean radiological follow-up among 137 patients was 26.0 ± 21.4 months. Of these, 43 patients (31.4%) experienced growth, with mean growth rate of 0.23 ± 4.7 mm/year; the remainder remained stable in size. Eight patients eventually underwent intervention (5 endovascular), with the most common indications including size criteria (4/8) and symptom development (3/8). No patient developed rupture. On multivariate analysis, obesity (P = .04) was significantly associated with growth. An initial diameter of more than 25 mm was significantly associated with subsequent intervention (P = .006), but was not significantly associated with growth. Four of five RAAs with an initial diameter 30 mm or greater did not undergo intervention. The mean clinical follow-up for these patients was 24 months; none developed rupture and two remained stable in size. CONCLUSIONS: This large institutional cohort found that the majority of RAAs remained stable in size, with few patients meeting indications for repair based on societal guidelines. Current guidelines recommending intervention for asymptomatic aneurysms more than 30 mm seem to be appropriate given their slow progression.

IRVAN syndrome: A retrospective review of 9 cases from Tunisia.

PURPOSE: To describe clinical features, relevant imaging findings, disease course, and response to treatment in 9 patients (18 eyes) with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Retrospective review of the charts of nine patients (18 eyes) diagnosed with IRVAN syndrome at Fattouma Bourguiba University Hospital, Monastir, Tunisia, from January 1, 2011 to January 1, 2022. RESULTS: Nine patients were included with bilateral involvement in all cases. Mean initial best-corrected visual acuity (VA) was 20/32 (range, 20/1600-20/20). Clinical findings at presentation included vitreous cells (10 eyes, 55.6%), peripapillary exudates (12 eyes, 66.7%), partial or complete macular star (11 eyes, 61.1%), and vascular sheathing (11 eyes, 61.1%). Fluorescein angiography showed arteriolar aneurysms (18 eyes, 100%), areas of peripheral capillary non-perfusion (16 eyes, 88.9%), and retinal neovascularization (6 eyes, 33.3%). Optical coherence tomography showed macular edema in 5 eyes (27.8%). Optical coherence tomography angiography of the optic disc demonstrated papillary aneurysms in 4 eyes of 2 patients. Indocyanine green angiography showed retinal arteriolar aneurysmal dilatations in 4 eyes of 2 patients. Ten eyes (55.6%) had stage 2 disease, 6 eyes (33.3%) had stage 3, and 2 eyes (11.1%) had stage 1. Treatment modalities included peripheral photocoagulation (16 eyes, 88.9%), intravitreal bevacizumab (4 eyes, 22.2%), and intravitreal triamcinolone acetonide (1 eye, 5.6%). Mean final best-corrected VA was 20/32 (range, 20/600-20/20). Ocular complications included vitreous hemorrhage in 3 eyes (16.7%), branch retinal artery occlusion in 2 eyes (11.1%) and submacular fibrosis in 3 eyes (16.7%). CONCLUSION: IRVAN syndrome should be highly suspected in patients with peripapillary exudates associated with vascular sheathing and vitreous cells. Multimodal imaging confirms the diagnosis by showing retinal macroaneurysms. Early treatment of macular edema and/or peripheral retinal non-perfusion is mandatory to improve prognosis.

Cohort Study Examining the Prevalence and Relationship with Outcome of Standard Modifiable Risk Factors in Patients with Peripheral Artery Occlusive and Aneurysmal Disease.

OBJECTIVE: The aim was to examine the presentation and outcome of patients with peripheral artery occlusive and aneurysmal disease (POAD) in relation to standard modifiable cardiovascular risk factors (SMuRFs; i.e., hypertension, diabetes, hypercholesterolaemia, and smoking). METHODS: A total of 2 129 participants with POAD were recruited from three vascular clinics in Queensland, Australia. SMuRFs were defined using established criteria. Participants were followed via outpatient appointments and linked data to record the primary outcome event of major adverse cardiovascular events (MACE). The association between SMuRFs and MACE was assessed using Cox proportional hazard analysis. Subanalyses examined the association of individual SMuRFs with MACE and assessed findings separately in participants with occlusive and aneurysmal disease. RESULTS: At recruitment 71 (3.3%), 551 (25.9%), 977 (45.9%), 471 (22.1%), and 59 (2.8%) participants had zero, one, two, three, and four SMuRFs. During a median follow up of 2.6 (interquartile range 0.4, 6.2) years, the risk of MACE was progressively higher with the increasing numbers of SMuRFs (adjusted hazard ratio [HR] 95% confidence interval [CI] 4.09, 1.29 - 12.91; 4.28, 1.37 - 13.41; 5.82, 1.84 - 18.39; and 9.42, 2.77 - 32.08; for one, two, three, or four SMuRFs, respectively) by comparison with those who were SMuRF-less at recruitment. Participants with occlusive disease were significantly more likely to have a greater number of SMuRFs than those with aneurysmal disease. In a subanalysis, there was a significantly higher risk of MACE with three or four SMuRFs in participants presenting with either occlusive or aneurysmal disease compared with those who were SMuRF-less. Hypertension, diabetes, and smoking but not hypercholesterolaemia were independently associated with increased risk of MACE. CONCLUSION: Very few patients presenting with POAD had no SMuRFs. There was a progressive increase in the risk of MACE in relation to the number of SMuRFs identified at entry.

Bronchial Artery Aneurysm: Prevalence, Clinical Characteristics, and Long-Term Prognosis Following Bronchial Artery Embolization.

PURPOSE: To investigate the prevalence, clinical characteristics, and long-term prognosis of bronchial artery aneurysm (BAA) following bronchial artery embolization (BAE). MATERIALS AND METHODS: The medical records of consecutive patients who underwent bronchial artery angiography between August 2013 and December 2019 were retrospectively reviewed. Patients who were diagnosed with BAA during this period were included in this study. The prevalence, patients' characteristics, symptoms, comorbidities, angiographic findings, and long-term prognosis following BAE were investigated. RESULTS: BAA was observed in 20 of 508 patients who underwent bronchial artery angiography (3.9%). The patients' median age was 69 (interquartile range [IQR], 63.5-76.7) years. The main causes of BAA were cryptogenic, bronchiectasis or cystic fibrosis, and pulmonary aspergillosis. The median diameter of ruptured BAAs was significantly smaller than that of unruptured BAAs (5.4 mm [IQR, 4.8-7.3 mm] vs 9.0 mm [IQR, 7.2-13.9 mm], P = .009). All the patients were successfully treated with BAE, without major adverse events. The median follow-up period after BAE was 970 (IQR, 561-1,796) days. The BAA-related survival rate was 100% at 2 and 3 years after BAE, and the overall survival rate after BAE was 89.2% (95% confidence interval [CI] 89.0-89.3) at 2 years and 74.3% (95% CI 74.0-74.5) at 3 years. BAA-related adverse events and mortality did not occur during the follow-up period. CONCLUSIONS: BAA was observed in 3.9 % (20/508) of the patients who underwent bronchial artery angiography. All the patients with BAA were successfully treated with BAE. BAA rupture and consequent mortality did not occur during the follow-up period.

Prevalence and Disease Spectrum of Extracoronary Arterial Abnormalities in Spontaneous Coronary Artery Dissection.

Importance: Spontaneous coronary artery dissection (SCAD) has been associated with fibromuscular dysplasia (FMD) and other extracoronary arterial abnormalities. However, the prevalence, severity, and clinical relevance of these abnormalities remain unclear. Objective: To assess the prevalence and spectrum of FMD and other extracoronary arterial abnormalities in patients with SCAD vs controls. Design, Setting, and Participants: This case series included 173 patients with angiographically confirmed SCAD enrolled between January 1, 2015, and December 31, 2019. Imaging of extracoronary arterial beds was performed by magnetic resonance angiography (MRA). Forty-one healthy individuals were recruited to serve as controls for blinded interpretation of MRA findings. Patients were recruited from the UK national SCAD registry, which enrolls throughout the UK by referral from the primary care physician or patient self-referral through an online portal. Participants attended the national SCAD referral center for assessment and MRA. Exposures: Both patients with SCAD and healthy controls underwent head-to-pelvis MRA (median time between SCAD event and MRA, 1 [IQR, 1-3] year). Main Outcome and Measures: The diagnosis of FMD, arterial dissections, and aneurysms was established according to the International FMD Consensus. Arterial tortuosity was assessed both qualitatively (presence or absence of an S curve) and quantitatively (number of curves ≥45%; tortuosity index). Results: Of the 173 patients with SCAD, 167 were women (96.5%); mean (SD) age at diagnosis was 44.5 (7.9) years. The prevalence of FMD was 31.8% (55 patients); 16 patients (29.1% of patients with FMD) had involvement of multiple vascular beds. Thirteen patients (7.5%) had extracoronary aneurysms and 3 patients (1.7%) had dissections. The prevalence and degree of arterial tortuosity were similar in patients and controls. In 43 patients imaged with both computed tomographic angiography and MRA, the identification of clinically significant remote arteriopathies was similar. Over a median 5-year follow-up, there were 2 noncardiovascular-associated deaths and 35 recurrent myocardial infarctions, but there were no primary extracoronary vascular events. Conclusions and Relevance: In this case series with blinded analysis of patients with SCAD, severe multivessel FMD, aneurysms, and dissections were infrequent. The findings of this study suggest that, although brain-to-pelvis imaging allows detection of remote arteriopathies that may require follow-up, extracoronary vascular events appear to be rare.

A systematic review and meta-analysis of treatment and natural history of popliteal artery aneurysms.

OBJECTIVE: To summarize the best available evidence comparing open vs endovascular popliteal artery aneurysm (PAA) repair. We also summarized the natural history of PAAs to support of the Society for Vascular Surgery guidelines. METHODS: We searched MEDLINE, EMBASE, Cochrane databases, and Scopus for studies of patients with PAAs treated with an open vs an endovascular approach. We also included studies of natural history of untreated patients. Studies were selected and appraised by pairs of independent reviewers. A meta-analysis was performed when appropriate. RESULTS: We identified 32 original studies and 4 systematic reviews from 2191 candidate references. Meta-analysis showed that compared with the endovascular approach, open surgical repair was associated with higher primary patency at 1 year (odds ratio [OR], 2.10; 95% confidence interval [CI], 1.41-3.12), lower occlusion rate at 30 days (OR, 0.41; 95% CI, 0.24-0.68) and fewer reinterventions (OR, 0.28; 95% CI, 0.17-0.45), but a longer hospital stay (standardized mean difference, 2.16; 95% CI, 1.23-3.09) and more wound complications (OR, 5.18; 95% CI, 2.19-12.26). There was no statistically significant difference in primary patency at 3 years (OR, 1.38; 95% CI, 0.97-1.97), secondary patency (OR, 1.59; 95% CI, 0.84-3.03), mortality at the longest follow-up (OR, 0.49; 95% CI, 0.21-1.17), mortality at 30 days (OR, 0.28; 95% CI, 0.06-1.36), or amputation (incidence rate ratio, 0.85; 95% CI, 0.56-1.31). The certainty in these estimates was, in general, low. Studies of PAA natural history suggest that thromboembolic complications and amputation develop at a mean observation time of 18 months and they are frequent. One study showed that at 5 years, approximately one-half of the patients had complications. CONCLUSIONS: This systematic review provides event rates for outcomes important to patients with PAAs. Despite the low certainty of the evidence, these rates along with surgical expertise and anatomic feasibility can help patients and surgeons to engage in shared decision-making.

The Society for Vascular Surgery clinical practice guidelines on popliteal artery aneurysms.

The Society for Vascular Surgery clinical practice guidelines on popliteal artery aneurysms (PAAs) leverage the work of a panel of experts chosen by the Society for Vascular Surgery to review the current world literature as it applies to PAAs to extract the most salient, evidence-based recommendations for the treatment of these patients. These guidelines focus on PAA screening, indications for intervention, choice of repair strategy, management of asymptomatic and symptomatic PAAs (including those presenting with acute limb ischemia), and follow-up of both untreated and treated PAAs. They offer long-awaited evidence-based recommendations for physicians taking care of these patients.

Prevalence of and Factors Associated with Arterial Aneurysms in Patients with Hereditary Hemorrhagic Telangiectasia: 17-Year Retrospective Series of 418 Patients.

PURPOSE: To estimate the prevalence of and identify characteristics associated with the presence of aneurysms in a cohort of patients with hereditary hemorrhagic telangiectasia (HHT). MATERIALS AND METHODS: In the study institution's HHT database, 418 patients with a definite HHT diagnosis were identified based on the clinical Curaçao criteria and/or an HHT-associated genetic mutation. Regression modeling was used to evaluate the association between arterial aneurysms and older age, male sex, smoking, alcohol consumption, hypertension, hyperlipidemia, genetic mutations, the presence of arteriovenous malformations (AVMs) unrelated to the aneurysms, and HHT-related genetic mutations. RESULTS: Forty-three (10.3%) patients had at least 1 aneurysm. Sixteen (3.8%) patients had multiple aneurysms. Of the variables analyzed, older age (odds ratio [OR] = 1.02; 95% confidence interval [CI]: 1.0-1.1), the presence of anatomically and flow-unrelated AVMs (OR = 3.2; 95% CI: 1.3-8.0), and the presence of activin A receptor type II-like 1 (ACVRL1) mutation (OR = 4.0; 95% CI: 1.5-10) were associated with the presence of at least 1 aneurysm. CONCLUSIONS: In this cohort of patients with HHT, the prevalence of intracranial and visceral arterial aneurysms was estimated to be 10.3%. Older age, the presence of unrelated AVMs, and the presence of the ACVRL1 mutation were associated with the presence of arterial aneurysms. Further study is required to assess the clinical importance and risk of rupture of aneurysms in patients with HHT.

Development and validation of an institutional nomogram for aiding aneurysm rupture risk stratification.

Rupture risk stratification is critical for incidentally detected intracranial aneurysms. Here we developed and validated an institutional nomogram to solve this issue. We reviewed the imaging and clinical databases for aneurysms from January 2015 to September 2018. Aneurysms were reconstructed and morphological features were extracted by the Pyradiomics in python. Multiple logistic regression was performed to develop the nomogram. The consistency of the nomogram predicted rupture risks and PHASES scores was assessed. The performance of the nomogram was evaluated by the discrimination, calibration, and decision curve analysis (DCA). 719 aneurysms were enrolled in this study. For each aneurysm, twelve morphological and nine clinical features were obtained. After logistic regression, seven features were enrolled in the nomogram, which were SurfaceVolumeRatio, Flatness, Age, Hyperlipemia, Smoker, Multiple aneurysms, and Location of the aneurysm. The nomogram had a positive and close correlation with PHASES score in predicting aneurysm rupture risks. AUCs of the nomogram in discriminating aneurysm rupture status was 0.837 in a separate testing set. The calibration curves fitted well and DCA demonstrated positive net benefits of the nomogram in guiding clinical decisions. In conclusion, Pyradiomics derived morphological features based institutional nomogram was useful for aneurysm rupture risk stratification.

Incidence, Diagnosis, Treatment, and Outcomes of True Superficial Artery Aneurysms.

BACKGROUND: True superficial femoral artery aneurysms (SFAAs) do not occur frequently but carry a high risk of limb loss when they are complicated with thrombosis, distal embolization or rupture. Large aneurysms can also exert a mass effect, compressing adjacent veins and nerves. We performed an updated review of the literature with regard to their incidence, diagnosis, treatment, and outcomes over the years. METHODS: A MEDLINE, Excerpta Medica Database (EMBASE) search of papers reporting SFAAs was conducted. Studies reported in the literature were considered for the review regardless of their nature and the number of participants. The available data regarding patient demographics, method of diagnosis, size, location, clinical presentation, therapy, and outcomes were examined. RESULTS: Searching from 1967 to the present, we identified 59 papers reporting true SFAAs. Forty-one papers were case reports with 1 case each, 9 papers with 2 cases each and 9 papers reporting case series with more than 3 cases. There was 1 study reporting data on the management of asymptomatic SFAAs. We identified 164 SFA aneurysms, and 84.4% were symptomatic. The mean age of patients at the presentation was 70.2 years (range, 27-96) and 89.4% were men. SFAAs were diagnosed earlier in patients with connective tissue disorders, with a mean age of 35 years (range, 29-43). In 60.2 % of cases are present symptoms related to mass compression, 26,8% with rupture, and 27,3% of cases with ischemic symptoms. Endovascular approach was chosen in 52.6% of SFAAs reported in the last 5 years. CONCLUSIONS: The data in the available literature are poor regarding the management of asymptomatic aneurysms, and the conclusions are based mainly on the symptomatic aneurysms reported. Most SFAAs present mainly with symptoms due to mass compression and rupture rather than ischemic symptoms. The endovascular approach is becoming more popular and could become the preferred choice.

Segmental overgrowth and aneurysms due to mosaic PDGFRB p.(Tyr562Cys).

Activating variants in the platelet-derived growth factor receptor ß gene (PDGFRB) have been associated with Kosaki overgrowth syndrome, infantile myofibromatosis, and Penttinen premature aging syndrome. A recently described phenotype with fusiform aneurysm has been associated with mosaic PDGFRB c.1685A > G p.(Tyr562Cys) variant. Few reports however have examined the vascular phenotypes and mosaic effects of PDGFRB variants. We describe clinical characteristics of two patients with a recurrent mosaic PDGFRB p.(Tyr562Cys) variant identified via next-generation sequencing-based genetic testing. We observed intracranial fusiform aneurysm in one patient and found an additional eight patients with aneurysms and phenotypes associated with PDGFRB-activating variants through literature search. The conditions caused by PDGFRB-activating variants share overlapping features including overgrowth, premature aged skin, and vascular malformations including aneurysms. Aneurysms are progressive and can result in morbidities and mortalities in the absence of successful intervention. Germline and/or somatic testing for PDGFRB gene should be obtained when PDGFRB activating variant-related phenotypes are present. Whole-body imaging of the arterial tree and echocardiography are recommended after diagnosis. Repeating the imaging study within a 6- to 12-month period after detection is reasonable. Finally, further evaluation for the effectiveness and safety profile of kinase inhibitors in this patient population is warranted.

Safety of Transcatheter Arterial Embolization for Visceral Artery Pseudoaneurysms: Incidence of Intraprocedural Rupture.

INTRODUCTION: Transcatheter arterial embolization is the first-line treatment for visceral artery pseudoaneurysms (VAPAs); however, the intraprocedural rupture of pseudoaneurysms is an important complication. The present study was performed to evaluate the safety of embolization for VAPAs, including the incidence of intraprocedural rupture. METHODS: Among 56 consecutive patients with 57 VAPAs who underwent treatment between April 2009 and October 2020, 46 patients with 47 VAPAs underwent embolization. Complications related to embolization including intraprocedural rupture, the technical success rate, and clinical outcomes were evaluated. Complications that required extended hospitalization, an advanced level of care, or resulted in permanent adverse sequelae or death were classified as major complications, while the remainder were considered to be minor. Technical success was defined as the completion of embolization. RESULTS: The intraprocedural rupture of pseudoaneurysms occurred in 3 out of 47 VAPAs treated with embolization (6%) and resulted in minor complications. One liver abscess requiring drainage was regarded as a major complication (2%). Focal infarction after embolization was observed as a minor complication in 20 cases. Complications occurred in 24 out of 47 cases (51%), comprising one major complication (2%) and 23 minor complications (48%). The technical success rate was 100% (47/47). Fifty-three out of 56 patients (95%) were alive in a median follow-up period of 18 months (range: 2 days-137 months). CONCLUSIONS: Embolization is safe and useful for the treatment of VAPAs; however, the intraprocedural rupture of pseudoaneurysms may occur, and, thus, care is needed during this procedure.

Popliteal artery aneurysms treatments: early midterm results of the use of endovascular stent grafts

Background/aim: Popliteal artery aneurysms (PAAs) are abnormal bulgings, which account for 70% of all peripheral artery aneurysms. They are usually asymptomatic. In this study, we present our long-term results of endovascular stent grafts in the treatment of PAA in the light of literature data. Material and methods: A total of 63 legs of 63 patients with PAA, who were treated with endovascular techniques in our clinic between July 2010 and July 2019, were retrospectively analyzed. All patients underwent color Doppler ultrasound (DUS), magnetic resonance angiography (MRA), or computed tomography angiography (CTA) to identify the diameter and length of PAAs, vessel tortuosity, the presence and degree of thrombus, and diameter in the healthy landing zone and to visualize tibioperoneal vascular structures. A Viabahn stent graft was inserted in all patients. Results: 57 patients (90.5%) were males with a mean age of 76.35 ± 7 years. 24 patients (38.1%) were symptomatic, while 11 patients (17.5%) had a concomitant abdominal aortic aneurysm (AAA). The mean follow-up period was 46.05 ± 25.01 months. The primary patency rate was 79.3%. A graft thrombosis was observed in 13 patients (20.6%) during a mean follow-up period of 8.31 ± 5.91 months. The number of distal arteries was significantly lower in the patients with thrombosis than those without. Conclusions: Endovascular treatment of PAA using stentgrafts is safe in selected cases. However, it is reasonable to avoid endovascular treatment due to an increased risk for thrombosis in patients with a low number of patent distal arteries or impaired distal flow.

Prevalence, growth rate and complications of splenic artery aneurysms in chronic liver disease patients.

Background: Chronic liver disease (CLD) patients are at greater risk for developing splenic artery aneurysm (SAA). Treatment for aneurysms > 2.5 cm in this population is considered. However, the procedure might be challenging in CLD patients, and complications may interfere in liver transplantation. We, therefore, sought to estimate the prevalence, growth rate and complications of SAA in patients with CLD. As secondary objective, we sought to evaluate whether those features differ in pre and post transplantation follow-up and among aneurysms with diameters greater or less than 2.5 cm at diagnosis. Patients and methods: We searched for the terms "SAA" and "CLD" on CT or MRI reports from January 2009 to December 2016. Patients with single examination or less than 6 months follow-up were excluded. Results: Fifty nine out of 2050 CLD patients presented SAA (prevalence of 2.9%). Fifteen patients were excluded (due to exclusion criteria). Forty-four CLD patients (mean age 55.9 years) presented 76 SAA (follow-up median of 27.2 months). Aneurysms presented mean size of 1.5 ± 0.74 cm at diagnosis and growth rate of 0.12 ± 0.14 cm/y. Two (4.5%) patients presented mild complications (aneurysm thrombosis). No significant differences were observed in the growth rates of aneurysms < 2.5 cm and ≥ 2.5 cm or in the initial size and growth rates of aneurysms of patients submitted to and not submitted to liver transplantation. Conclusions: The estimated prevalence of SAA in patients with DLC in the Brazilian population is 2.9% (CI95% 2.2-3.6%). Although SAA in CLD patients are less likely to remain stable and grow faster than in general population, aneurysms are usually diagnosed at smaller size and complications are rare. These findings might support conservative management with close surveillance, especially in smaller aneurysms.

Systematic Review of the Co-Prevalence of Arterial Aneurysms Within the Vasculature.

OBJECTIVE: Aneurysms are considered focal manifestations of a systemic vascular condition, and various studies report co-prevalence of aneurysms in different vascular beds. Insight into profiles of patients at risk of multiple aneurysms is lacking, and few clinical algorithms exist if additional screening is indicated. This systematic review assessed the co-prevalence of aneurysms in different vascular beds and analysed putative risk factors for multiple aneurysms. METHODS: Medline, Embase, and Cochrane libraries were searched up to February 2020 for studies reporting co-prevalence of aneurysms in different vascular beds using the keywords: "aneurysm", "co-prevalence", or synonyms. All studies were reviewed by two authors independently. Studies were excluded if they described concomitant treatment of multi-aneurysms, or if the aneurysm was reported solely bilateral, post-dissection, mycotic, traumatic, iatrogenic, or caused by a connective tissue disease. Radar plots were used to indicate studies that found an association between the investigated features and aneurysm co-prevalence against those that did not. RESULTS: Thirty-two studies met the inclusion criteria, describing in total 16 353 patients of whom 2 015 had at least one additional aneurysm. The weighted co-prevalence was 16.9% (95% confidence interval [CI] 11.8-22.6), I2 > 90%. At least 19 combinations of aneurysms were described, mostly derived from retrospective studies. Seventeen of 32 (53%) studies described concurrent aneurysms in patients with an abdominal aortic aneurysm. Predominantly positive associations were found for higher age, hypertension, stenotic disease, presence of multiple (at least three) aneurysms, and primary aneurysm size. CONCLUSION: Approximately one in six patients with a primary aneurysm harbours an additional aneurysm, increasing to one in four if the patient has a popliteal artery aneurysm. Higher age, hypertension, stenotic disease, presence of multiple (at least three) aneurysms, and primary aneurysm size were predictive of aneurysm co-prevalence. These clinical predictors may assist when deciding whether a patient with a primary aneurysm needs to be screened for additional aneurysms.

Contemporary Treatment of Popliteal Artery Aneurysms in 14 Countries: A Vascunet Report.

OBJECTIVE: Popliteal artery aneurysm (PAA) is the second most common arterial aneurysm. Vascunet is an international collaboration of vascular registries. The aim was to study treatment and outcomes. METHODS: This was a retrospective analysis of prospectively registered population based data. Fourteen countries contributed data (Australia, Denmark, Finland, France, Hungary, Iceland, Italy, Malta, New Zealand, Norway, Portugal, Serbia, Sweden, and Switzerland). RESULTS: During 2012-2018, data from 10 764 PAA repairs were included. Mean values with between countries ranges in parenthesis are given. The incidence was 10.4 cases/million inhabitants/year (2.4-19.3). The mean age was 71.3 years (66.8-75.3). Most patients, 93.3%, were men and 40.0% were active smokers. The operations were elective in 73.2% (60.0%-85.7%). The mean pre-operative PAA diameter was 32.1 mm (27.3-38.3 mm). Open surgery dominated in both elective (79.5%) and acute (83.2%) cases. A medial surgical approach was used in 77.7%, and posterior in 22.3%. Vein grafts were used in 63.8%. Of the emergency procedures, 91% (n = 2 169, 20.2% of all) were for acute thrombosis and 9% for rupture (n = 236, 2.2% of all). Thrombosis patients had larger aneurysms, mean diameter 35.5 mm, and 46.3% were active smokers. Early amputation and death were higher after acute presentation than after elective surgery (5.0% vs. 0.7%; 1.9% vs. 0.5%). This pattern remained one year after surgery (8.5% vs. 1.0%; 6.1% vs. 1.4%). Elective open compared with endovascular surgery had similar one year amputation rates (1.2% vs. 0.2%; p = .095) but superior patency (84.0% vs. 78.4%; p = .005). Veins had higher patency and lower amputation rates, at one year compared with synthetic grafts (86.8% vs. 72.3%; 1.8% vs. 5.2%; both p < .001). The posterior open approach had a lower amputation rate (0.0% vs. 1.6%, p = .009) than the medial approach. CONCLUSION: Patients presenting with acute ischaemia had high risk of amputation. The frequent use of endovascular repair and prosthetic grafts should be reconsidered based on these results.

Higher prevalence of splenic artery aneurysms in hereditary hemorrhagic telangiectasia: Vascular implications and risk factors.

BACKGROUND: Splenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT. METHODS: We analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement. RESULTS: SAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03-4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02-1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01-4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF. CONCLUSIONS: We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.