Screening chest, abdomen, and pelvis CTA in patients with FMD showed substantial and incremental diagnostic yield. Reformatted images should routinely be included in imaging analysis. Abnormalities in the aorta were not common, so screening of the thoracic aorta may not be indicated.

Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.

Purpose: To analyze the relationship between the new TransAtlantic Inter-Society Consensus (TASC II) infrapopliteal classification and limb outcomes among patients with critical limb ischemia (CLI). Methods: A single-center retrospective study was performed on 166 consecutive CLI patients (mean age 71 years; 113 men) undergoing endovascular treatment of 244 infrapopliteal lesions from 2006 to 2013. Patient, procedural, angiographic, and limb outcomes were compared for the new TASC A/B vs C/D classification for infrapopliteal lesions. Binary restenosis was determined by a peak systolic velocity ratio >2.0 by duplex ultrasound on follow-up at 1, 3, 6, and 12 months. Results: Seventy-two (43.4%) patients had TASC A/B lesions, while 94 (56.6%) had TASC C/D patterns of infrapopliteal disease. Baseline demographics and tissue loss (93% vs 94%, p=0.59) were similar between the groups. TASC A/B lesions were shorter (53±35 vs 170±83 mm, p<0.001), less severely stenosed (77%±24% vs 93%±14%, p<0.001), had a larger target vessel diameter (2.9±0.5 vs 2.6±0.5 mm, p<0.001), and were less frequently chronic total occlusions (24% vs 64%, p<0.001) compared with the TASC C/D group. Threeyear freedom from both amputation (85% vs 67%, p=0.02) and major adverse limb events (79% vs 61%, p=0.02) were significantly higher in the TASC A/B group. Technical success rates (95% vs 80%, p<0.001) and 1-year primary patency (58% vs 51%, p=0.04) were higher in the A/B group. Overall 3-year survival was similar between the groups (96% A/B vs 88% C/D, p=0.2). Conclusion: TASC C/D infrapopliteal lesions are associated with higher amputation and major adverse limb events rates and lower primary patency compared with TASC A/B infrapopliteal lesions. Further studies are needed to assess the association between TASC C/D infrapopliteal lesions and clinical outcomes in patients with CLI.

Ankle-brachial indices (ABIs) are important for the assessment of disease burden among patients with peripheral artery disease. Although low values have been associated with adverse clinical outcomes, the association between non-compressible ABI (ncABI) and clinical outcome has not been evaluated among patients with critical limb ischemia (CLI). The present study sought to compare the clinical characteristics, angiographic findings and clinical outcomes of those with compressible (cABI) and ncABI among patients with CLI. Consecutive patients undergoing endovascular evaluation for CLI between 2006 and 2013 were included in a single center cohort. Major adverse cardiovascular events (MACE) were then compared between the two groups. Among 284 patients with CLI, 68 (24%) had ncABIs. These patients were more likely to have coronary artery disease ( p=0.003), diabetes ( p<0.001), end-stage renal disease ( p<0.001) and tissue loss ( p=0.01) when compared to patients with cABI. Rates of infrapopliteal disease were similar between the two groups ( p=0.10), though patients with ncABI had lower rates of iliac ( p=0.004) or femoropopliteal stenosis ( p=0.003). Infrapopliteal vessels had smaller diameters ( p=0.01) with longer lesions ( p=0.05) among patients with ncABIs. After 3 years of follow-up, ncABIs were associated with increased rates of mortality (HR 1.75, 95% CI: 1.12-2.78), MACE (HR 2.04, 95% CI: 1.35-3.03) and major amputation (HR 1.96, 95% CI: 1.11-3.45) when compared to patients with cABIs. In conclusion, ncABIs are associated with higher rates of mortality and adverse events among those undergoing endovascular therapy for CLI.

Background Fibromuscular Dysplasia (FMD) is an non-atherosclerotic disease associated with hypertension, headache, dissection, stroke, and aneurysm. The etiology is unknown but hypothesized to involve genetic and environmental components. Previous studies suggest a possible overlap of FMD with other connective tissue diseases that present with dissections and aneurysms. The aim of this study was to investigate the prevalence of connective tissue physical features in FMD. Methods and Results 142 FMD patients were consecutively enrolled at a single referral center (97.9% female, 92.3% had multifocal FMD). Data are reported for 139 female patients. Moderately severe myopia (29.1%), high palate (33.1%), dental crowding (29.7%), and early onset arthritis (15.6%) were prevalent features. Classic connective features such as hypertelorism, cleft palate, and hypermobility were uncommon. Frequency of systemic connective tissue features was compared between FMD patients with a high vascular risk profile (having had ≥1 dissection and/or ≥2 aneurysms) and those with a standard vascular risk profile. History of spontaneous pneumothorax (5.9% high risk vs. 0% standard risk) and atrophic scarring (17.3% high risk vs. 6.8% standard risk) were significantly more prevalent in the high risk group, p<0.05. High palate was observed in 43.1% of the high risk group vs. 27.3% in the standard risk group, p=0.055. Conclusions In a cohort of women with FMD, there was a prevalence of moderately severe myopia, high palate, dental crowding, and early onset osteoarthritis. However, a characteristic phenotype was not discovered. Several connective tissue features such as high palate and pneumothorax were more prominent among FMD patients with a high vascular risk profile.

Objective Accreditation for noninvasive vascular testing facilities has been available since 1990, but is not mandatory. We sought to determine the perceived value of accreditation among the staff of vascular laboratories accredited by the Intersocietal Accreditation Commission (IAC). Methods A multi-item electronic survey was sent to medical and technical staff and administrative contacts within the IAC database. Respondents were asked to rate statements about the impact of accreditation on their facility. Data for vascular testing facilities are presented. Results Of the 7,289 surveys sent, there were 882 (12.1%) respondents. Respondents were primarily responsible for the facility's accreditation application (75.0%), with the majority being technologists (82.0%), followed by physicians (11.3%). Most respondents were from hospital-based facilities (51.1%) and from facilities accredited for >3 years (79.6%). The majority, 94.3% of respondents, felt that maintaining accreditation of their facility was important (3.5% neutral, 2.2% not important). The greatest perceived benefits were in standardization of study acquisition and reporting, adherence to guidelines, and report completeness. Conclusions The majority of respondents from IAC-accredited vascular testing facilities viewed accreditation favorably. The favorable perception of accreditation by its users supports the value of accreditation for vascular testing facilities.

Presenting symptoms for patients with UE FMD vary in severity from asymptomatic disease to digital ischemia. More than half of symptomatic limbs eventually require at least one invasive intervention for complete relief of symptoms.

Fibromuscular dysplasia (FMD), a disease well described in the renal and cerebrovascular circulations, also manifests in the lower extremity (LE) arteries. This study reports on the clinical presentation, imaging findings, and treatment of patients with LE FMD seen at a single center. Over a 7-year span, 100 of 449 patients with FMD had imaging of the LE arteries, of which 62 were found to have LE FMD (13.8% of the entire FMD cohort including patients with and without LE imaging). The majority of patients were women (96.8%), with an average age of 52 ± 11.3 years at the time of diagnosis. All patients had FMD present in another vascular bed, most commonly in the renal (80.6%) and extracranial carotid arteries (79.0%). Most patients had multifocal FMD (95.2%) and bilateral LE disease (69.4%), with the external (87.1%), common (19.4%), and internal (11.3%) iliac arteries most commonly affected. Presenting symptoms of LE involvement included claudication (22.6%), atypical leg symptoms (14.5%), and dissection (6.5%), but most patients were asymptomatic (71.0%). Nearly all patients were managed conservatively (98.4%) and only 1 patient required intervention.