Ellen Brinza

Fibromuscular dysplasia: Advances in understanding and management.

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.

Systemic connective tissue features in women with fibromuscular dysplasia

Fibromuscular dysplasia (FMD) is a 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. A total of 142 FMD patients were consecutively enrolled at a single referral center (97.9% female, 92.1% of whom 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. The 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. A history of spontaneous pneumothorax (5.9% high risk vs 0% standard risk) and atrophic scarring (17.6% 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 versus 27.3% in the standard risk group, p=0.055. In conclusion, 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.

Accreditation is Perceived to Improve the Quality of Vascular Testing Facilities

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 facilitys 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.

Presentation, clinical features, and results of intervention in upper extremity fibromuscular dysplasia

Background: We present a case series of upper extremity fibromuscular dysplasia (UE FMD) consisting of 22 patients from two tertiary referral centers focusing on clinical presentation, diagnostic findings, and interventional outcomes. FMD is a noninflammatory, nonatherosclerotic arteriopathy that has a predisposition for middle‐aged women. Involvement of the UE is thought to be rare. Patients with UE FMD can present with claudication or ischemia, or they can be incidentally diagnosed. The treatment approach is dictated by clinical presentation. Methods: Data were collected of patients with UE FMD evaluated at two centers. Demographic data, presenting UE symptoms, UE arteries involved, FMD type, diagnostic method, physical examination findings, management, and outcomes were included. Results: Twenty‐two patients (29 limbs) were diagnosed with UE FMD. The brachial artery was most commonly involved (89.7% of affected limbs). More than half of limbs (n = 15 of 29 limbs [51.7%]) were asymptomatic, and of those who presented with symptoms, the most common symptoms were ischemic fingers or hand (31% of all affected limbs) and hand or arm claudication (27.6% of all affected limbs). UE FMD was noted on catheter angiography in 58.6% (n = 17 of 29 limbs), duplex ultrasound in 41.4% (n = 12 of 29 limbs), and computed tomography angiography in 27.6% (n = 8 of 29 limbs). Of the symptomatic limbs (n = 14), the majority were treated solely with medical therapy as the first intervention (57.1%). For symptomatic limbs treated with vascular intervention (n = 5), angioplasty was most commonly performed. Only 4 of the 14 limbs (28.6%) had complete symptomatic relief after the initial first intervention, in which 2 limbs were treated with medical therapy, 1 limb underwent angioplasty, and 1 limb had resolution of symptoms despite deferment of any therapy. Of the 10 limbs with residual symptoms after the first intervention, 6 limbs underwent a second intervention: angioplasty in 2 limbs initially treated medically (33.3%), surgical bypass in 2 limbs initially treated with angioplasty, surgical bypass in 1 limb initially treated with medical therapy, and sympathectomy in 1 limb (16.7%) initially treated with angioplasty. Both surgical bypass and angioplasty as secondary interventions resulted in complete symptom relief. Conclusions: 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.

Midterm Outcomes after Infrapopliteal Interventions in Patients with Critical Limb Ischemia Based on the TASC II Classification of Below-the-Knee Arteries

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. Three-year 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.

Lower Extremity Fibromuscular Dysplasia: Clinical Manifestations, Diagnostic Testing, and Approach to Management

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.

FAMILY HISTORY OF ARTERIAL DISORDERS IN PATIENTS WITH SPONTANEOUS CORONARY ARTERY DISSECTION

Background: Spontaneous coronary dissection (SCAD) is an important cause of ACS in young women and is associated with fibromuscular dysplasia (FMD) in 45-72% of cases. ∼1/4 of FMD patients have a family history (FHx) of aneurysm, but FHx of arterial disorders among SCAD patients is not well