Moyamoya Vasculopathy and Moyamoya-Related Systemic Vasculopathy: A Review With Histopathological and Genetic Viewpoints.

Systemic vasculopathy has occasionally been reported in cases of moyamoya disease (MMD). Since the pathological relationship between moyamoya vasculopathy (MMV) and moyamoya-related systemic vasculopathy (MMRSV) remains unclear, it was examined herein by a review of histopathologic studies in consideration of clinicopathological and genetic viewpoints. Although luminal stenosis was a common finding in MMV and MMRSV, histopathologic findings of vascular remodeling markedly differed. MMV showed intimal hyperplasia, marked medial atrophy, and redundant tortuosity of the internal elastic lamina, with outer diameter narrowing called negative remodeling. MMRSV showed hyperplasia, mainly in the intima and sometimes in the media, with disrupted stratification of the internal elastic lamina. Systemic vasculopathy has also been observed in patients with non-MMD carrying the RNF213 (ring finger protein 213) mutation, leading to the concept of RNF213 vasculopathy. RNF213 vasculopathy in patients with non-MMD was histopathologically similar to MMRSV. Cases of MMRSV have sometimes been diagnosed with fibromuscular dysplasia. Fibromuscular dysplasia is similar to MMD not only in the histopathologic findings of MMRSV but also from clinicopathological and genetic viewpoints. The significant histopathologic difference between MMV and MMRSV may be attributed to a difference in the original vascular wall structure and its resistance to pathological stress between the intracranial and systemic arteries. To understand the pathogeneses of MMD and MMRSV, a broader perspective that includes RNF213 vasculopathy and fibromuscular dysplasia as well as an examination of the 2- or multiple-hit theory consisting of genetic factors, vascular structural conditions, and vascular environmental factors, such as blood immune cells and hemodynamics, are needed.

Risks and benefits of renal artery stenting in fibromuscular dysplasia: Lessons from the ARCADIA-POL study.

INTRODUCTION: Although renal stenting is the standard revascularization method for atherosclerotic renal artery stenosis (RAS) (FMD-RAS), stenting in fibromuscular dysplasia (FMD) RAS is usually limited to periprocedural complications of angioplasty and primary arterial dissection. The main aim of the study was to retrospectively analyze the immediate and long-term results of renal stenting versus angioplasty in patients with FMD. METHODS: Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries). Percutaneous transluminal renal angioplasty (PTRA) was performed as an initial treatment in 61 arteries (PTRA-group), whereas primary stenting was undertaken in nine arteries (stent-group). Stent-related complications were defined as: in-stent restenosis > 50% (ISR); stent fracture; under-expansion; or migration. RESULTS: In the PTRA-group, the initial restenosis rate was 50.8%. A second procedure was then performed in 22 arteries: re-PTRA (12 arteries) or stenting (10 arteries). The incidence of recurrent restenosis after re-PTRA was 41.7%. Complications occurred in seven of 10 (70%) arteries secondarily treated by stenting: two with under-expansion and five with ISR. In the stent-group, stent under-expansion occurred in one case (11.1%) and ISR in three of nine stents (33.3%). In combined analysis of stented arteries, either primarily or secondarily, stent-related complications occurred in 11/19 stenting procedures (57.9%): three due to under-expansion and eight due to ISRs. Finally, despite several revascularization attempts, four of 19 (21%) stented arteries were totally occluded and one was significantly stenosed at follow-up imaging. CONCLUSION: Our study indicates that renal stenting in FMD-RAS may carry a high risk of late complications, including stent occlusion. Further observational data from large-scale registries are required.

Cerebral arteriopathies of childhood and stroke - A focus on systemic arteriopathies and pediatric fibromuscular dysplasia (FMD).

Systemic vascular involvement in children with cerebral arteriopathies is increasingly recognized and often highly morbid. Fibromuscular dysplasia (FMD) represents a cerebral arteriopathy with systemic involvement, commonly affecting the renal and carotid arteries. In adults, FMD diagnosis and classification typically relies on angiographic features, like the 'string-of-beads' appearance, following exclusion of other diseases. Pediatric FMD (pFMD) is considered equivalent to adult FMD although robust evidence for similarities is lacking. We conducted a comprehensive literature review on pFMD and revealed inherent differences between pediatric and adult-onset FMD across various domains including epidemiology, natural history, histopathophysiology, clinical, and radiological features. Although focal arterial lesions are often described in children with FMD, the radiological appearance of 'string-of-beads' is highly nonspecific in children. Furthermore, children predominantly exhibit intimal-type fibroplasia, common in other childhood monogenic arteriopathies. Our findings lend support to the notion that pFMD broadly reflects an undefined heterogenous group of monogenic systemic medium-or-large vessel steno-occlusive arteriopathies rather than a single entity. Recognizing the challenges in categorizing complex morphologies of cerebral arteriopathy using current classifications, we propose a novel term for describing children with cerebral and systemic vascular involvement: 'cerebral and systemic arteriopathy of childhood' (CSA-c). This term aims to streamline patient categorization and, when coupled with advanced vascular imaging and high-throughput genomics, will enhance our comprehension of etiology, and accelerate mechanism-targeted therapeutic developments. Lastly, in light of the high morbidity in children with cerebral and systemic arteriopathies, we suggest that investigating for systemic vascular involvement is important in children with cerebral arteriopathies.

Migraine headache in patients with spontaneous coronary artery dissection: A report of the iSCAD Registry.

INTRODUCTION: Spontaneous coronary artery dissection (SCAD) is a nonatherosclerotic cause of myocardial infarction. Migraine headache has been reported to be common among patients with SCAD, but the degree of migraine-related disability has not been quantified. METHODS: Clinical data and headache variables were obtained from the baseline assessment of the prospective, multicenter iSCAD Registry. Migraine-related disability was quantified using the self-reported Migraine Disability Assessment (MIDAS). Demographic, clinical, psychosocial, and medical characteristics from data entry forms were compared between patients with and without migraine. RESULTS: Of the 773 patients with available data, 46% reported previous or current migraines. Those with migraines were more likely to be women (96.9% vs 90.3%, p = 0.0003). The presence of underlying carotid fibromuscular dysplasia was associated with migraine (35% vs 27%, p = 0.0175). There was not a significant association with carotid artery dissection and migraine. Current migraine frequency was less than monthly (58%), monthly (24%), weekly (16%), and daily (3%). Triptan use was reported in 32.5% of patients, and 17.5% used daily migraine prophylactic medications. Using the MIDAS to quantify disability related to migraine, 60.2% reported little or no disability, 14.4% mild, 12.7% moderate, and 12.7% severe. The mean MIDAS score was 9.9 (mild to moderate disability). Patients with SCAD had higher rates of depression and anxiety (28.2% vs 17.7% [p = 0.0004] and 35.3% vs 26.7% [p = 0.0099], respectively). CONCLUSIONS: Migraines are common, frequent, and a source of disability in patients with SCAD. The association between female sex, anxiety, and depression may provide some insight for potential treatment modalities.

Reversible Cerebral Vasoconstriction Syndrome And Fibromuscular Dysplasia: An Epiphenomenon Or A Causal Relationship?

Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a nonatheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months. While most RCVS cases have identifiable triggers, a significant portion occurs without known causes. Recent studies have reported a high prevalence of neurovascular abnormalities in RCVS patients. We present a case of a thirty-year-old woman with a sudden-onset severe headache, diagnosed with RCVS associated with carotid web. The patient had no ischemic involvement and responded well to flunarizine treatment. Follow-up imaging showed no stenosis. This case highlights a potential association between carotid web and RCVS, suggesting that FMD may contribute to vascular hyperreactivity and presents as a risk factor for RCVS. Further investigations are needed to understand the underlying mechanisms connecting these two vascular disorders. Keywords: reversible vasoconstriction syndrome; fibromuscular dysplasia; carotid web; structural abnormalities; vascular hyperreactivity.

Outcomes of carotid stenting in patients with fibromuscular dysplasia.

OBJECTIVE: Fibromuscular dysplasia (FMD) is a noninflammatory arterial disease that affects the extracranial carotid arteries in young patients. The ideal treatment of FMD has continued to be debated, and the role of carotid artery stenting (CAS) is controversial. The aim of the present study was to assess the feasibility and outcomes of CAS for patients with FMD. METHODS: A retrospective analysis of patients who had undergone CAS was performed using the Vascular Quality Initiative database from December 2012 to May 2021. Patients who had undergone CAS for atherosclerosis and FMD were included and matched 1:1 by age, gender, and clinical presentation. The demographics, clinical parameters, and procedural data were analyzed. The end points included postoperative stroke and transient ischemic attack (TIA), and adverse events (perioperative and 1-year mortality, neurologic changes, access site complications, hematoma or bleeding, infection, congestive heart failure, arrhythmia, myocardial infarction, reperfusion symptoms), and hospital length of stay. RESULTS: After matching, 55 patients had undergone CAS for FMD (mean age, 58.7 ± 14 years; 62% women; 69% White; mean body mass index, 28 ± 6 kg/m2). Most of these procedures (69%) were elective. The FMD group had had a lower rate of hypertension (55% vs 82%; P = .002), smoking (35% vs 80%; P < .001), diabetes (13% vs 45%; P < .001), and coronary artery disease (9% vs 45%; P < .001) compared with the non-FMD group. In the FMD group, prior TIA and stroke was identified in 39 (71%) and 31 (57%) patients, respectively. The mean interval from a prior stroke or TIA to the index surgery was 160 days. Additionally, 23 patients (42%) had had anatomically high lesions above the level of the second cervical vertebra. In the FMD group, the transfemoral approach was used for 43 patients (78%), with distal embolic protection used for 40 patients (93%). Flow reversal was used for nine patients (23%). Most cases were performed with local anesthesia (58%). Three patients (6%) in the FMD group had had access site complications that were managed nonoperatively. No differences were found between the FMD and non-FMD groups in perioperative stroke, TIA, or 30-day mortality. The length of stay was similar between the two groups, and the 1-year survival was 100% for both groups. All the patients in the FMD group were discharged without neurologic complications, and 50 patients (91%) were receiving dual antiplatelet therapy. The median follow-up was 328 days (interquartile range, 1-732 days) with no mortality or reinterventions during follow-up. CONCLUSIONS: CAS for FMD is a feasible and safe procedure with favorable technical success, a low incidence of neurologic complications, and good clinical outcomes at 1 year of follow-up.

The Effect of Endovascular Treatment of Renal Artery Stenoses on Coexistent Aneurysms Associated with Fibromuscular Dysplasia.

PURPOSE: Endovascular data on patients with coexistent renal artery stenosis (RAS) and renal artery aneurysm (RAA) caused by fibromuscular dysplasia (FMD) are scarce, and the outcomes from RAS-specific treatment on RAA remain unclear. This study aimed to evaluate the safety and effectiveness of RAS-specific endovascular management in patients with coexisting RAA caused by FMD. MATERIALS AND METHODS: Clinical and endovascular data on 19 patients with coexistent RAS and RAA caused by FMD who underwent RAS-specific endovascular therapy were analyzed prospectively. An RAA located within 10 mm of the RAS was defined as a stenosis-related RAA (SRAA), and long-term outcomes were evaluated. RESULTS: Nineteen patients (24 RASs and 30 RAAs) underwent endovascular therapy. Twenty-one RASs were treated with balloon angioplasty alone, whereas 3 RASs were treated with stent implantation. None of the RAAs were treated directly. During an average of 4.2 years ± 3.2 of follow-up, systolic and diastolic blood pressures decreased from 183.0 mm Hg ± 19.5 and 120.2 mm Hg ± 19.0 to 127.9 mm Hg ± 10.3 and 80.9 mm Hg ± 6.9, respectively; the number of antihypertensive medications reduced from 1.7 ± 1.0 to 0.8 ± 0.3 (for all, P < .001). The serum creatinine level remained stable. The maximum diameter of all RAAs decreased from 14.6 mm ± 9.7 to 11.3 mm ± 8.4 (P < .001). There was a significant difference in the improvement rate of the maximum diameter between SRAAs (65.0%, 13 of 20) and non-SRAAs (20.0%, 2 of 10) (P = .019). CONCLUSIONS: RAS-specific endovascular therapy is safe and effective and possibly aids in preventing RAA progression in patients with FMD with coexistent RAS and RAA.

Transient ischemic attack due to dynamic evolution of carotid artery web.

Carotid artery web (CaW) is a rare focal fibromuscular dysplasia, which is an underappreciated risk factor for transient ischemic attack. This case illustrates the dynamic evolution of secondary thrombus and plaque in CaW, and the importance of carotid doppler ultrasound in early detection and follow-up.

A Retrospective Cohort Study of Cerebrovascular Fibromuscular Dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD) is a rare vasculopathy for which limited data are available particularly from Europe. Our aim was to study the clinical characteristics of a regional cohort of carotid fibromuscular dysplasia patients to assess their clinical outcomes and the rate of vascular complications. METHODS: A retrospective cohort study of all cases of carotid/cerebrovascular FMD presenting to our regional vascular service (catchment population approximately 2 million), between 1998 and 2020. Imaging reports and patient case notes were screened using the keywords "FMD", "Fibromuscular Dysplasia", and "carotid". From case-note and imaging review, all relevant clinical data were extracted and the anatomical extent of vascular disease recorded. RESULTS: Eighty six patients with a diagnosis of cerebrovascular fibromuscular dysplasia were identified on imaging (31 computed tomography angiography, 46 magnetic resonance angiography, and 9 digital subtraction angiography) by a neurovascular radiologist. The mean age was 64 years, 78 (90%) patients were female, and 45/59 (75%) were Caucasian. Presenting clinical syndromes were Stroke/transient ischemic attack in 54 (63%) patients, symptomatic intracranial aneurysm in 6 (10%), and other neurological symptoms (headache/migraine, tinnitus) in 14 (16%), with 11 (13%) presenting incidentally. Six patients (7%) had a positive family history of FMD (2 patients) or other cerebrovascular event (4 patients: carotid dissection, intracerebral bleed, or stroke). Eight patients (9%) had a known or suspected hereditary connective tissue disorder (2 Ehlers-Danlos syndrome). Involved vessels were as follows: Carotid (mainly extracranial) in 79 (92%), vertebral 19 (22%), and a combination of these in 15 (17%) patients. Fifty eight (67%) patients had bilateral disease. Cerebrovascular complications were observed in 35 (41%) patients as follows: carotid dissection 11 (23%), carotid stenosis or occlusion 8 (9%), carotid aneurysm 8 (9%), cerebral aneurysm 9 (11%), vertebral aneurysm/dissection 2 (2%), and carotid-cavernous fistula 2 (2%). Of the 22 patients who had extracranial imaging, 14 (60%) had FMD affecting other beds-renal artery in 8 (36%) patients, other visceral arteries in 4 (18%), and aorta in 2 (9%). In addition, 4 (18%) patients had aneurysm or dissection affecting renal, splenic, and lower limb arteries. Overall, 67 (80%) patients had FMD affecting more than 1 vessel and 50 (58%) had multisite FMD (>/ = 2 vascular beds involved). Fifty nine (68%) patients were managed conservatively on close surveillance. Nineteen (21%) patients required carotid/cerebrovascular intervention and 9 (10%) required vascular intervention at other sites. Recurrent cerebrovascular events (stroke/transient ischemic attack, symptomatic Berry aneurysm) were seen in 20 (23%) patients. Overall mortality was 7% over a median follow-up period of 47 months. CONCLUSIONS: Carotid FMD patients have a high rate of multisite involvement, extracerebral vascular complications, and evidence of hereditary vasculopathy, requiring careful screening and surveillance.

Carotid Web Phenotype Is Uncommonly Associated With Classic Fibromuscular Dysplasia: A Retrospective Observational Study.

BACKGROUND AND PURPOSE: Carotid web (CaW) is an intimal form of fibromuscular dysplasia (FMD) involving the carotid bulb which has been increasingly recognized as a potential cause of recurrent ischemic strokes. It is overlooked as a separate entity and often dismissed if no coexistent signs of classic FMD changes are observed. We aim to evaluate the frequency of classic FMD in high-yield vascular territories in patients with symptomatic CaW. METHODS: This was a retrospective analysis of a symptomatic CaW database of 2 comprehensive stroke centers (spanning September 2014-October 2020). The diagnosis of a CaW during a stroke workup was defined as the presence of a shelf-like linear filling defect in the posterior aspect of the carotid bulb on computed tomography angiography in patients with acute ischemic stroke or transient ischemic attack of undetermined cause after a thorough evaluation. Neck computed tomography angiography and renal conventional angiography images were independently evaluated by two readers blinded to the laterality and clinical details to inspect the presence of underlying classic FMD. RESULTS: Sixty-six patients with CaW were identified. Median age was 51 years (interquartile range, 42-57), and 74% were women. All patients had neck computed tomography angiography (allowing for bilateral vertebral and carotid evaluation), whereas 47 patients had additional digital subtraction angiography (which evaluated 47 carotids ipsilateral to the stroke and 10 contralateral carotids). Internal carotid artery classic FMD changes were noted in only 6 out of 66 (9%) in the ipsilateral carotids. No contralateral carotid or vertebral artery classic FMD changes were observed. Renal artery catheter-based angiography was obtained in 16 patients/32 arteries and only 1 patient/2 renal arteries demonstrated classic FMD changes. CONCLUSIONS: CaW phenotype is uncommonly associated with classic FMD changes. Coexistent classic FMD does not constitute a useful marker to corroborate or exclude CaW diagnosis.

Insights from intravascular pressure measurement of renal artery revascularization in patients with fibromuscular dysplasia: The DYSART study.

OBJECTIVE: The indication of percutaneous renal transluminal angioplasty (PTRA) in fibromuscular dysplasia (FMD) is mainly based on renal artery stenosis (RAS) due to atherosclerosis criteria, which are not specific to FMD. Consequently, the selection of patients who could benefit from this treatment and its effectiveness remain uncertain. The aims of this study were to: (1) report the effects of PTRA guided by trans-stenotic pressure measurements on hypertension 7 months after treatment; (2) assess the impact of pressure measurement to guide treatment efficacy in comparison to visual angiographic parameters; and (3) evaluate the reproducibility and accuracy of the stenosis measurement using a 4F catheter in comparison to a pressure guidewire. METHODS: This prospective multi-centric study analyzed 24 patients with hypertension with RAS due to FMD that required PTRA. Clinical, duplex ultrasound, and angiographic indices were collected, and patients were followed up for 7 months (±1 month). Angiographic indices were measured twice both by a pressure guidewire and a 4F catheter. Assessment of procedural and clinical success of angioplasty was performed for all patients. RESULTS: Twenty-three patients (96%) had procedural success (considered as a post-PTRA translesional systolic gradient ≤10 mmHg or reduced by at least 80%) with a significant decrease in the systolic gradient after angioplasty (26.50 mmHg; [interquartile range, 16.75-38.75] vs 0.00 [interquartile range, 0.00-2.00]; P < .01). Three patients (12%) had complications, including two renal artery dissections and one partial renal infarction. Twenty-one patients (88%) were clinical responders to angioplasty at follow-up. Visual stenosis assessment showed a poor correlation with systolic gradient measurement before and after PTRA (R from -0.05 to 0.41; P = 0.06-0.82). High correlations were found between pressure measurements made by a 4F catheter and guidewire (R from 0.64 to 0.89; P ≤ .003). CONCLUSIONS: In patients selected by clinical indicators and duplex ultrasound, reaching a translesional systolic gradient ≤10 mmHg or reduced by at least 80% after angioplasty, promotes a high success rate for PTRA in hypertension due to FMD RAS.

The complex genetic basis of fibromuscular dysplasia, a systemic arteriopathy associated with multiple forms of cardiovascular disease.

Artery stenosis is a common cause of hypertension and stroke and can be due to atherosclerosis accumulation in the majority of cases and in a small fraction of patients to arterial fibromuscular dysplasia (FMD). Artery stenosis due to atherosclerosis is widely studied with known risk factors (e.g. increasing age, male gender, and dyslipidemia) to influence its etiology, including genetic factors. However, the causes of noninflammatory and nonatherosclerotic stenosis in FMD are less understood. FMD occurs predominantly in early middle-age women, a fraction of the population where cardiovascular risk is different and understudied. FMD arteriopathies are often diagnosed in the context of hypertension and stroke and co-occur mainly with spontaneous coronary artery dissection, an atypical cause of acute myocardial infarction. In this review, we provide a comprehensive overview of the recent advances in the understanding of molecular origins of FMD. Data were obtained from genetic studies using complementary methodological approaches applied to familial, syndromic, and sporadic forms of this intriguing arteriopathy. Rare variation analyses point toward mechanisms related to impaired prostacyclin signaling and defaults in fibrillar collagens. The study of common variation, mainly through a recent genome-wide association study, describes a shared genetic link with blood pressure, in addition to point at potential risk genes involved in actin cytoskeleton and intracellular calcium homeostasis supporting impaired vascular contraction as a key mechanism. We conclude this review with future strategies and approaches needed to fully understand the genetic and molecular mechanisms related to FMD.

Pediatric Renal Artery Stenosis: A 19-Year Experience in Management and Outcomes at a Tertiary Pediatric Hospital.

BACKGROUND: Renal artery stenosis (RAS) is an uncommon cause of pediatric hypertension. Guidelines for workup and management have not been established. The most widely reported etiology of the pediatric renovascular disease has been fibromuscular dysplasia; however, other etiologies including middle aortic syndrome (MAS) and vasculitides have been described. We reviewed cases of radiologically identified pediatric RAS and describe etiologies, management, and long-term clinical outcomes in our patients. METHODS: Reports for duplex ultrasound, computed tomography angiography, magnetic resonance imaging, and conventional angiography from an academic children's hospital between 2000 and 2019 were evaluated. Positive reports for RAS were confirmed by a vascular surgeon and a radiologist. Demographics, indications for evaluation, management, and long-term clinical outcomes were documented. Data are summarized as count (n), geometric mean, median, or standard deviation as appropriate. Univariate differences between treatment cohorts were analyzed using Chi-squared tests for categorical variables. Nonparametric paired Wilcoxon signed-rank test and Mann-Whitney U-test were used for the analysis of paired ordinal or continuous data. A statistical analysis was performed with SPSS software (SPSS Inc., Chicago, IL) with significance defined at a P < 0.05 level. RESULTS: Imaging for suspected RAS was performed on 984 children. Of the 38 patients with positive imaging for RAS, 60.5% were idiopathic, 31.5% (n = 12) had concomitant congenital/systemic comorbidity, and 21.0% (n = 8) had RAS and concomitant aortic pathology. Fibromuscular dysplasia only accounted for 13.2% (n = 5) of patients. Regarding management, 34.2% (n = 13) underwent invasive intervention, 23.7% (n = 9) underwent endovascular intervention alone, and 10.5% (n = 4) underwent endovascular plus surgical intervention. Conservative management was performed for 65.8% (n = 25) of patients at a long-term follow-up (33.8 months), 34.2% (n = 13) requiring only lifestyle changes, and 31.6% (n = 12) requiring only medical management. CONCLUSIONS: Pediatric RAS is a low-frequency disease and long-term outcomes have been under-reported. The incidence of associated aortic pathology in our intervention cohort appears higher than that was previously reported. A long-term follow-up demonstrated that up to 65.8% of patients could be managed successfully with conservative therapy.

Outcomes Following the Endovascular Treatment of Renal Artery Stenosis Caused by Fibromuscular Dysplasia: A Systematic Review and Meta-Analysis.

OBJECTIVE: Renal artery revascularization has been performed to improve blood pressure control and to cure hypertension in patients with renal artery fibromuscular dysplasia (RAFMD). We herein conducted a systematic review and meta-analysis of studies assessing outcomes associated with the treatment of hypertensive RAFMD patients via endovascular angioplasty in order to offer an up-to-date overview of the relative costs and benefits of this approach to revascularization in RAFMD patients. METHODS: We systematically searched the PubMed and Embase databases and the Cochrane Central Register for Controlled Trials to identify relevant studies published as of January 15, 2020. Key outcomes of interest in these studies included technical success, the incidence of perioperative complications, cure rates, and overall improvement rates. RESULTS: In total, we identified 36 relevant studies of 1916 total repairs conducted in 1191 patients. Of these included studies, 33 were retrospective, while 3 were prospective. The overall technical success rate across these studies was 94.3%. Rates of total, major, and minor complications in these pooled studies were 12.9%, 4.6%, and 7.4%, respectively. Pooled rates of cured hypertension and improved hypertension following angioplasty, defined according to study-specific criteria, were 37.0% [95% CI: 27.0%-47.0%] and 80.0% [95% CI: 75.0% to 84.0%], respectively, although these rates varied highly among studies. Cure rates for studies used current clinical definitions for substantial variations across studies. Cure rates in studies using current definitions of cured hypertension (blood pressure <140/90 mm Hg without treatment) were just 18.1% following angioplasty. Cure rates fell markedly with increasing mean patient age (OR associated with an increase in mean age of 10 years: -0.24 [95% CI: -0.44 to -0.04, P = 0.019] and with mean known duration of hypertension (OR associated with an increase in mean hypertension duration of 5 years: -0.09 [95% CI: -0.12 to -0.05, P = 0.001]). CONCLUSIONS: These findings suggest that endovascular treatment yielded moderate benefits to RAFMD patients, with substantial variation across studies. The blood pressure outcome was strongly influenced by patient age.

Retrograde Recanalization for Proximal Occlusion of the Right Renal Artery through a Compensated Collateral Artery in a 10-year-old Patient.

BACKGROUND: To describe a retrograde recanalization for the proximal occluded lesion in right renal artery (RRA) in young patient with fibromuscular dysplasia (FMD). METHODS: A 10-year-old girl presented to our hospital with proximal RRA occlusion and refractory hypertension though she took anti-hypertension medicines. Her renin and aldosterone were beyond the normal level in both base state and excited state. Her glomerular filtration rate at right kidney was only 18.4 ml/min. Angiography revealed proximal RRA occlusion and a compensated collateral artery (CCA) from the infrarenal aorta to the RRA. She was thus diagnosed with focal FMD. A retrograde recanalization was performed through this CCA. RESULTS: Angioplasty and stenting were successfully performed to treat the proximal RRA occlusion. Postoperatively, the glomerular filtration rate in the right kidney improved. One-year follow-up revealed that, the blood pressure maintained at normal range without any antihypertensive agents. No other discomfort was complained. CONCLUSIONS: It is feasible to establish a working pathway with patient's compensated collateral artery to treat the renal artery occlusion.

Renal Artery Stenosis due to Entrapment.

BACKGROUND: Common etiologies of renovascular occlusive disease include atherosclerosis disease, developmental fibrotic conditions such as fibromuscular dysplasia, and vasculitis. Extrinsic compression of the renal artery is a rarely reported phenomenon but can lead to similar clinical manifestations. METHODS: We report recent experience with 2 patients who presented with extrinsic renal artery compression due to entrapment. Diagnosis was made with a constellation of findings on computed tomography angiography, dynamic duplex sonography, and catheter angiography. Both patients had hypertension and 1 had downstream subsegmental renal infarcts. The patients, both with right-sided renal artery entrapment, were treated with open surgical decompression. Exposure was achieved via extended Kocher maneuver followed by mobilization of the right kidney and, in 1 patient, detachment of the right lobe of liver to allow circumferential exposure of the proximal right renal artery to the aorta. All entrapping tissue was circumferentially released. RESULTS: Both operations were uncomplicated. Intraoperative sonography was used to confirm luminal patency of the released segments. Follow-up of renal artery duplex in both patients demonstrated resolution of dynamic compression. Renal artery peak systolic velocity and accelerations indices were all within normal limits. In both patients, improvement in blood pressure control was noted and discontinuation of anticoagulation was possible in the patient who had recurrent episodes of renal infarct. CONCLUSIONS: Extrinsic compression of renal artery by diaphragmatic crura is rare but should be considered in younger patients or otherwise any patients with no vascular risk factors when renovascular hypertension workup yields no demonstrable intrinsic disease. A high index of suspicion should be raised when an anomalously high origin of the renal artery or proximity to the diaphragmatic crura is seen on cross-sectional imaging. Work-up should include dynamic imaging to assess compression of renal arteries during expiration. Open surgical or laparoscopic decompression of the involved renal arteries can be curative.

The Relationship Among Pain Location, Complications, and Quality of Life in Individuals With Fibromuscular Dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD), a nonatherosclerotic arterial disease, can cause pain and vascular complications. The aim of this study was to examine the impact of FMD symptoms and complications on quality of life, depression, anxiety, and self-rated health. DESIGN: This was a cross-sectional, correlational study. METHODS: Participants were adults with a diagnosis of FMD. Quality of life (36-Item Short Form Health Survey), anxiety and depression (Patient-Reported Outcomes Measurement Information System [PROMIS®]), self-rated health question, and symptom/complication questionnaires were mailed to patients with FMD. Scores were compared with symptoms and complications. Multivariable linear models were fit for symptoms and survey scores. Ordinal regression was used for self-rated health. Backwards selection was run for each model. Alpha of 0.05 and 95% confidence intervals were used. RESULTS: Of the 162 (275 total; 47.8%) patients who returned surveys (156 female), 130 had carotid or vertebral artery involvement (80.2%). Migraine (p < .001), neck pain (p = .036), and flank pain (p = .025) were associated with decrease in Mental Component scores. Migraine (p = .002) and neck pain (p = .023) were associated with lower Physical Component scores. Patients reporting abdominal pain compared with those without had 4.88 points higher depression. Abdominal pain (p = .031) and pulsatile tinnitus (p = .011) were associated with greater anxiety. Migraine was associated with (p = .002) lower self-rated health. Participants with history of stroke/transient ischemic attack had 2.42 (1.08, 5.46; p = .033) times the odds of poor self-rated health compared with those without stroke/transient ischemic attack. CONCLUSIONS: Among patients with FMD, presence of pain and history of vascular complications were related to lower quality of life and self-rated health.

Coronary fibromuscular dysplasia with three-vessel involvement: a rare cause of ischaemic dilated cardiomyopathy in a very young male.

Coronary artery disease of non-atherosclerotic aetiology, while rare in incidence, can have a wide aetiology, such as fibromuscular dysplasia, which is a non-inflammatory arteriopathy of numerous histopathological types of fibromuscular tissue accumulation. This brief report describes the case of a 22-year-old male with a recently developed dilated cardiomyopathy and a history of aborted cardiac arrest at the age of 14 years. Coronary angiogram revealed severe three vessels disease, while optical coherence tomography established fibromuscular dysplasia as aetiology. Balloon and stent angioplasty was performed guided by fractional flow reserve with acceptable angiographic result.

[Management of the patient with renal artery fibromuscular dysplasia: clinical case].

Fibromuscular dysplasia (FMD) is a rare disease that affects small and medium-sized arteries. Clinical manifestations of FMD depend on its localization. In many cases, FMD of renal arteries (RA) is associated with arterial hypertension. Young age, particularly of female patients, suspected RA dissection or kidney infarction, absence of atherosclerosis or presence of FMD in other arteries of such patients evidence for RA FMD. In invasive treatment of hemodynamically significant stenoses, transluminal balloon angioplasty (TBA) of renal arteries is preferrable. Taking into account initial alterations of the vascular wall and unevenness of the lumen of the affected blood vessel, stent implantation is associated with an increased risk of complications and is recommended only if ballooning complications develop. An open reconstructive surgery is indicated in complicated narrowing anatomy, a high risk of the endovascular treatment, or after failure of the endovascular intervention. This article presents a clinical case of a young female patient with RA FMD and renovascular arterial hypertension who successfully underwent renal TBA with a drug-coated balloon.

[Arterial fibromuscular dysplasia as a factor in sudden cardiac death in young adults]. / Fibromuskulyarnaya displaziya arterii ­ odin iz faktorov vnezapnoi serdechnoi smerti lits molodogo vozrasta.

The objective of the study is to consider the problem of diagnostics of a rare vasculopathy, fibromuscular dysplasia of coronary arteries, by the case study of a 19-year-old serviceman, an athlete with sudden death occurred during slight physical exertion. After repeated histological examination as part of the forensic medical examination, the diagnosis was made: «multifocal fibromuscular dysplasia of the coronary arteries and ascending aortic arch, complicated by acute left ventricular failure.¼ This disease often manifests with the acute coronary syndrome in people of young age. Therefore, special attention was given to the macroscopic and histological features of fibromuscular dysplasia of arteries.