Prevalence Of Fibromuscular Dysplasia Research Articles

Coronary Artery Tortuosity and Spontaneous Coronary Artery Dissection: Association With Echocardiography and Global Longitudinal Strain, Fibromuscular Dysplasia, and Outcomes

The etiology and significance of coronary artery tortuosity (TCA) among patients with spontaneous coronary artery dissection (SCAD) are unknown. The aim of this prospective imaging cohort study was to report echocardiographic findings and evaluate whether TCA correlates with cardiac anatomy and function among patients with SCAD. Comorbidities including fibromuscular dysplasia (FMD) and outcomes were also assessed. TCA was determined on coronary angiography performed during the diagnosis of SCAD, and cardiac structure and function were evaluated using prospective comprehensive echocardiography. Among 116 patients with SCAD, the mean age at echocardiography was 50.8±8.8years, a median of 10.9months after SCAD. Sixty-two patients (53.4%) had FMD, 41 (35.3%) had histories of hypertension, and 17 (14.8%) were hypertensive during echocardiography. Most patients (n=78 [69%]) had normal left ventricular geometry with normal median ejection fraction (61%; interquartile range, 56% to 64%) and normal global longitudinal strain (-22.2%; interquartile range, -24.0% to -19.9%). Fifteen patients (13.4%) had diastolic dysfunction that was associated with hypertension at the time of echocardiography. Patients with TCA (n=96 [82.8%]) were older (mean age, 52.1±8.0 vs 44.7±9.9years; P<.001) with a higher prevalence of FMD (59.4% vs 25%, P=.007) but a similar prevalence of hypertension (35% vs 35%, P>.99) compared with patients without TCA. Across the age range (31.5 to 66.9years), each decade of age was associated with an approximately 0.89-unit increase in coronary tortuosity score (P<.0001). Echocardiographic parameters were not significantly different between the two groups. Median follow-up duration was 4.4years (95% CI, 3.8 to 5.2years). The Kaplan-Meier 3-year SCAD recurrence rate was 9.4% (95% CI, 3.7% to 14.8%). There were no deaths. The majority of patients with SCAD had normal or near normal echocardiographic results, including global longitudinal strain, with no differences according to TCA. However, patients with SCAD with TCA were older, with a higher prevalence of FMD.

Characteristics and Prognosis of Patients With Fibromuscular Dysplasia in a Population of Spontaneous Coronary Artery Dissections (from the French Registry of Spontaneous Coronary Artery Dissections “DISCO”)

Spontaneous coronary artery dissection (SCAD) and fibromuscular dysplasia (FMD) are pathologies that appear to be closely related. This study compares the characteristics of the FMD population to the non-FMD population in a SCAD cohort. It thus assesses the involvement of the FMD phenotype in a SCAD population. From the data of the French DISCO registry, we included patients with a diagnosis of SCAD and in whom a search for FMD was performed. We collected the following characteristics of this population: the clinical and angiographic presentation, the data concerning the management, and the events occurring during the follow-up. In the 373 SCADs confirmed in the DISCO registry, we obtained imaging data for 340 of them. FMD was found in 45% of cases. The mean age was higher in the FMD group, 53.2 ± 8.8 years, versus 50.1 ± 11 years in the non-FMD group. High blood pressure and postmenopausal status were significantly higher in the FMD group. Clinical presentation, angiographic data, and management were comparable. The major adverse cardiac event rate and recurrence rate were not different between the 2 groups after 1 year of follow-up. In conclusion, we confirmed a 45% prevalence of FMD in the SCAD population. The median age was higher in the FMD group, suggesting that FMD may develop over time. The rate of major adverse cardiac events and recurrence were similar in the FMD group versus the non-FMD group after 1 year of follow-up.

Фибромышечная дисплазия в клинической практике

This article provides information on the prevalence of fibromuscular dysplasia and its role in the development of arterial hypertension, as well as gives the classification, summarizes approaches to diagnosis and treatment, describes cases of different localization of fibromuscular dysplasia and individual (personalized) management tactics for this category of patients.

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

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. To assess the prevalence and spectrum of FMD and other extracoronary arterial abnormalities in patients with SCAD vs controls. 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. Both patients with SCAD and healthy controls underwent head-to-pelvis MRA (median time between SCAD event and MRA, 1 [IQR, 1-3] year). 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). 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. 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.

VERTEBRAL ARTERY DISSECTION AND INCIDENTAL FINDING OF FIBROMUSCULAR DYSPLASIA: A CASE REPORT

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Fibromuscular Dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease. It is a rare condition that occurs more frequently in middle-aged Caucasian women. Its prevalence is not well known. We present a case of FMD in a man with stroke secondary to dissection of the vertebral artery. Increased reporting of FMD cases in recent studies raises concern about under-diagnosis of the condition. CASE PRESENTATION: A 61-year-old Caucasian male with a past medical history of hypertension presented with dysarthria, extreme disequilibrium, and left-sided headache. The patient had normal strength and sensation in all four extremities and negative cerebellar testing on physical exam. He also had a productive cough upon presentation. Computed tomography (CT) head revealed a 3 cm acute non-hemorrhagic left cerebellar infarction. Computed tomography angiography (CTA) Head and Neck showed evidence of vertebral artery dissection on the left side, including the origin of the left posterior inferior cerebellar artery (PICA). The patient had a trial of cerebral angiogram for the vertebral artery dissection, which showed incidental evidence of FMD in the femoral arteries bilaterally. The patient also had vasospasm with the angiography that resolved post-injection of five milligrams of Verapamil intraarterial, and the procedure was discontinued. He was discharged on aspirin and statin, and anticoagulation starting in two weeks. DISCUSSION: There is no clear consensus on the prevalence of FMD in the general population [1]. However, many studies have assessed the prevalence in different patient populations to be around 3%-4% [2]. The United States Registry of FMD revealed the incidence of dissection of the carotid artery in the FMD patient population to be about 75% [1]. The incidence of dissection of the vertebral artery in patients diagnosed with FMD is much lower, about 17% [1]. The most common manifestations of cervical artery dissection (CeAD) are cerebral infarction and transient ischemic attack (TIA). In FMD patients with ischemic stroke or TIA secondary to CeAD, general guidelines for thrombolysis and thrombectomy should be followed. Most recent guidelines recommend treatment with either an anticoagulant or an antiplatelet agent for at least 3–6 months [3]. Endovascular or surgical repairs are mainly reserved for patients with recurrent ischemia despite initial antithrombotic therapy. CONCLUSIONS: FMD is an intriguing vascular disorder with unclear underlying etiology, challenging manifestations, and complex clinical diagnosis. Increased reporting of FMD cases in recent literature raises concern for underdiagnosis. Furthermore, in our case, FMD was incidentally discovered during a diagnostic procedure to evaluate the vertebral artery dissection. Therefore, we recommend further screening of any patient who presents with cephalo-cervical dissection or aneurysm for underlying FMD. REFERENCE #1: Olin, J.W., Froehlich, J., Gu, X., et al. 2012, "The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients", Circulation, vol. 125, no. 25, pp. 3182-3190. REFERENCE #2: Hendricks, N.J., Matsumoto, A.H., Angle, J.F., et al. 2014, "Is fibromuscular dysplasia underdiagnosed? A comparison of the prevalence of FMD seen in CORAL trial participants versus a single institution population of renal donor candidates", Vascular medicine (London, England), vol. 19, no. 5, pp. 363-367. REFERENCE #3: Powers, W.J., Rabinstein, A.A., Ackerson, et al. "2018 Guidelines for the Early Management of Patients With Acute Ischemic Stroke: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association", Stroke, vol. 49, no. 3, pp. e46-e110. DISCLOSURES: No relevant relationships by Elizabeth Mause, source=Web Response No relevant relationships by Mohammad Selim, source=Web Response

PREVALENCE AND NATURE OF EXTRA-CORONARY ARTERIAL ABNORMALITIES IN PATIENTS WITH SPONTANEOUS CORONARY ARTERY DISSECTION VS. CONTROLS

Abstract Objective: Spontaneous Coronary Artery Dissection (SCAD) has been associated with a high prevalence of Fibromuscular Dysplasia (FMD) and other extra-coronary arterial abnormalities. However, the prevalence, severity and clinical relevance of these abnormalities is still unclear. The objectives of the current study were: (i) to assess extra-coronary arterial abnormalities by MRA in patients with SCAD compared to controls; (ii) to compare the performance of MRA vs. CTA. Design and method: All images were jointly analysed by a vascular medicine specialist and a radiologist blinded for subject status and characteristics. In addition to qualitative analysis of images, arterial tortuosity was assessed by number of angles &gt; 45% and tortuosity index in the main vascular beds. Results: MRA from brain to pelvis was available for 166 patients with SCAD (97% women, age at diagnosis: 44.4 ± 7.8 years) and 42 healthy controls matched for age and gender. The prevalence of FMD in patients with SCAD was 30.7% vs. 9.5% in controls (p = 0.005). In the SCAD group, the most frequently affected vascular beds were renal (13.9%), cerebrovascular (12.0%) and iliac (10.8%) arteries. Only 22% of SCAD patients had FMD lesions in 2 or more arterial beds. Nine SCAD patients (5.4%) had aneurysms vs. one (2.4%) in the control group (p = 0.5). No case of arterial dissection was identified. The prevalence and degree of arterial tortuosity was similar in cases and controls, irrespective of measurement method or arterial bed. Finally, in 35 patients explored both by CTA and MRA, the performance of MRA for detection of extra-coronary arterial abnormalities was similar to that of CTA. Conclusions: Based on the current blinded analysis of a large series of fully explored patients with SCAD compared to controls: (i) extra-coronary FMD is three times more frequent in patients with SCAD than in apparently healthy controls; (ii) FMD associated with SCAD is less often multivessel and associated with less severe arterial manifestations than “classical” FMD; (iii) patients with SCAD are not prone to extra-coronary arterial tortuosity; (iv) the performance of MRA is similar to that of CTA for detection of extra-coronary abnormalities in patients with SCAD.

Prevalence and characteristics of renal artery fibromuscular dysplasia in hypertensive women below 50 years old

Renal fibromuscular dysplasia (FMD) is typically diagnosed in young hypertensive women. The 2014 European FMD Consensus recommended screening in all hypertensive women <30 yo. However, the prevalence of renal FMD in young/middle-aged hypertensive women remains unclear. The aim of this work was to assess the prevalence and characteristics of renal FMD in hypertensive women ≤50 yo. We retrospectively included all consecutive women aged ≤50years referred to our Hypertension Unit from 2014 to 2017 and collected standardized information on patient characteristics and screening modalities. Of 1083 incident hypertensive patients, 157 patients fitted with inclusion criteria. The prevalence of renal FMD varied between 3.2% in the whole sample and 7.5% in patients explored by CTA and/or MRA (n=67). In the subgroup of patients ≤30 yo (n=32), the corresponding figures were 3.1% and 5.6%. The yearly prevalence of FMD tended to increase over time, in parallel with increased use of CTA/MRA as a first-line imaging modality. Out of 5 patients with renal FMD, 2 were revascularized and 1 had extra-renal FMD. The prevalence of renal FMD in young/middle-aged hypertensive women is probably one order of magnitude higher than previously assumed, in the range of 3%-8%, depending on imaging modalities. While the diagnosis of FMD does not influence short-term management in all patients, it may allow close monitoring and prevention of complications of the disease over time. This analysis provides the rationale for a prospective, multicentre study aiming at determining the cost-effectiveness of systematic screening for renal FMD.

Prevalence, Presentation, and Associated Conditions of Patients With Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) is defined by focal narrowing of small and medium-sized arteries due to an idiopathic, noninflammatory, nonatherosclerotic vascular disease. The population-based prevalence of FMD remains unknown. Using the National Inpatient Sample database, we evaluated the prevalence, clinical presentation, mortality, and associated conditions of FMD from January 1, 2004, to September 30, 2015. Among 2,420 patients who presented with FMD, 2,086 (86.20%) of patients were female. The mean age was 55.18 ± 18.99 years in men and 63.37 ± 17.10 years in women. FMD patients most commonly presented with hypertension (67.3%), transient ischemic attack (3.7%), headache (2.1%), dizziness (1.1%), abdominal pain (0.6%), or hematuria (0.3%). In-hospital mortality of FMD patients was 0.74%. In conclusion, FMD is a rare condition with low in-hospital mortality that may be considered among female patients presenting with hypertension.

FEASIBILITY OF ESTIMATING FIBROMUSCULAR DYSPLASIA PREVALENCE USING HEALTH ADMINISTRATIVE DATABASE

Objective: Fibro-muscular dysplasia (FMD) is a non-atherosclerotic and non-inflammatory vascular disease, occurring mainly in women aged 30–50 years. Its prevalence is not well-known and possibly underestimated due to asymptomatic forms. Our aim was to determine whether the French national summary discharge database could be used to estimate the prevalence of FMD. Design and method: Patients were selected by the first occurrence of the I773 ICD-10 code. This code was chosen after an exploratory analysis and advice from national hypertension specialists. In the French national summary discharge database system, all hospitalizations can be linked for a given patient. The zip code of residence was used for geographical analysis. We computed rates of prevalence by an indirect age- and sex-standardization. Results: Between 2008 and 2016, we identified 2798 patients (65.2% female; median age 55y IQR = [43.0–67.0]), for a total of 10835 stays (74.9% of patients had less than 2 stays) of which only 4061 contained the I773 code. No major change in annual incident cases was observed between 2008 to 2016: 317 (11.3%), 342 (12.2%), 292 (10.4%), 309 (11.0%), 299 (10.7%), 319 (11.4%), 294 (10.5%), 279 (9.97%) and 347 (12.4%), respectively. The most common associated codes were I10 (hypertension, 49.0%), I15.0 (vascular/renal hypertension, 19.7%) and E780 (essential hypercholesterolemia, 11.9%). Among all French hospitals, the largest number of patients was identified in the Paris University Hospitals, especially ESH excellence centers (15.7%) and 8 additional University Hospitals belong to the ’top 10’ recruiting centres. Using indirect standardization, we observed a geographical disparity with a low prevalence in the East/South-West part of France, which may indicate under-diagnosis of the disease and a very high unexplained prevalence in a single hospital.Conclusions: Both the stable estimate of yearly incident cases and demographic characteristics of the population study are consistent with the literature. Nevertheless, the observed geographical disparity suggests a diagnostic bias. We conclude that the use of the French national summary discharge database alone cannot provide a reliable estimation of the FMD prevalence. Both harmonization of diagnosis procedures and hospitalization coding could help identifying FMD patients in addition to existent multicentric registries.

Fibromuscular dysplasia: what the radiologist should know: a pictorial review.

Fibromuscular dysplasia (FMD) is an idiopathic, segmentary, non-inflammatory and non-atherosclerotic disease that can affect all layers of both small- and medium-calibre arteries. The prevalence of FMD is estimated between 4 and 6 % in the renal arteries and between 0.3 and 3 % in the cervico-encephalic arteries. FMD most frequently affects the renal, carotid and vertebral arteries, but it can theoretically affect any artery. Radiologists play an important role in the diagnosis of FMD, and good knowledge of FMD’s signs will certainly help reduce the delay between the first symptoms and diagnosis. The common string-of-beads aspect is well known, but less common presentations also have to be considered. These less common imaging findings include vascular loops, fusiform vascular ectasia, arterial dissection, aneurysm and subarachnoid haemorrhage. These radiologic presentations should be known by radiologists in order to diagnose possible FMD, particularly when present in young females or when associated with personal or familial hypertension, to reduce the delay between the onset of the first symptom and the final diagnosis. The patients have to be referred to specialised FMD centres for dedicated management.Teaching Points• Fibromuscular dysplasia is not a rare disease. • Radiologists should recognise less common presentations to orient specific management. • Vascular loops, fusiform vascular ectasia and a “string-of-beads” aspect are typical presentations. • Arterial dissection, aneurysm and subarachnoid haemorrhage are less typical radiologic presentations.

Treatment of renal fibromuscular dysplasia in an adolescent male: A case report

Introduction: Fibromuscular dysplasia (FMD) is a rare vascular disease that causes stenosis in the major arteries such as the renal and carotid arteries. The exact prevalence of FMD in the general population is not known. It is often presented with symptoms of hypertension. Mainly reported in middle-aged females, the diagnosis of FMD is often delayed. There are very few case reports of FMD in males and children in literature. Case Report: We report the case of a 13-year-old asymptomatic boy found to have FMD in the right renal artery (RRA). An angiography revealed high grade stenosis that was corrected by surgery. A hepatorenal bypass using anastomosis of the gastroduodenal artery (GDA) to RRA was deemed appropriate. An angioplasty at the anastomosis six weeks after this procedure corrected an upward trend in postsurgical blood pressure readings and RRA blood velocities. Two years after the surgery, the patient has well-controlled blood pressure, managed with low dose of ACE-inhibitors. Conclusion: Hepatorenal bypass of RRA through anastomosis of GDA was a viable surgical option in this case. The monitoring of blood pressure readings allowed for early detection and correction of any post-surgical stenosis.

Insights of Optical Coherence Tomography in Renal Artery Fibromuscular Dysplasia in a Patient with Spontaneous Coronary Artery Dissection

A 60-year-old woman was admitted for an acute coronary syndrome. Coronary angiography showed a spontaneous coronary artery dissection (SCAD) in the left anterior descending coronary artery (A). A typical image of fibromuscular dysplasia (FMD) was also observed in the right renal artery (B). Optical coherence tomography (OCT) (C) revealed alternating areas of thickening and thinning of the medial layer, corresponding to the typical image of string of beads readily identified in the longitudinal reconstruction of the OCT and also in angiography. A very high prevalence of FMD in non-coronary arteries has been recently reported in patients with DCE. Our findings suggest that OCT may provide unique diagnostic clues in these challenging patients.

8:48 AM Abstract No. 18 - ■ FEATURED ABSTRACT Is fibromuscular dysplasia (FMD) underdiagnosed? A comparison of the prevalence of fmd seen in patients enrolled into the CORAL trial versus a single institution population of renal donor candidates

Purpose We postulate that FMD is underdiagnosed. Therefore, we evaluated the prevalence of renal artery FMD in patients enrolled into the CORAL renal artery stent trial, in which the presence of renal artery FMD was an exclusion criterion for trial enrollment. We also evaluated the prevalence of FMD in a relatively healthy population of patients undergoing screening CT angiography (CTA) for comparison. Materials and Methods Following IRB approval, we did a retrospective review of renal donor preoperative CTAs performed and interpreted by fellowship trained cardiovascular imaging physicians at our institution between 1/2003 and 11/2011. These results were then compared to the findings from the angiographic core lab (ACL) for the CORAL renal artery stent trial in which patients with FMD were theoretically excluded from trial enrollment. Data was assessed for the presence of renal artery FMD and the patient’s age and gender. Results The CORAL ACL database contained 1014 patients (mean age = 69.4 years) and there were 504 males (49.7%) and 510 females (50.3%). Fifty-eight CORAL trial patients (5.7%) were found to have incidental renal artery FMD (95% CI [4.4, 7.35]). The mean age of these 58 patients was 71.8 years, and 44 were female (75.9%). The preoperative kidney donor population included 220 consecutive patients (mean age = 40.5 years). There were 78 males (35.5%) and 142 females (64.5%). Five patients (2.3%) had evidence of FMD. The mean age of these 5 patients was 48.6 years and all 5 (100%) were female. Conclusion The prevalence of renal artery FMD of 2.3% is consistent with prior reports (1,2). However, the prevalence of FMD of 5.7% in the more elderly CORAL population (which was biased against the selection of patients with FMD) suggests that FMD is underdiagnosed in patients with atherosclerotic renal artery stenosis. The female predilection for renal FMD remains in patients of 70 years of age.

A unique case of renal artery fibromuscular dysplasia

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disorder resulting in arterial stenosis. Here, a unique case of renal artery FMD (RA FMD) is presented. With the use of angiograpic and intravascular ultrasound (IVUS) imaging modalities, a patient was diagnosed with two distinct types of FMD. Until now, the prevalence of FMD has been underestimated due to limitations in diagnostic modalities. Clinically, the correct identification of RA FMD is essential because these patients have been shown to respond to revascularization therapy with significant improvement in blood pressure whereas renal artery lesions secondary to other etiologies such as atherosclerosis have yet to show the same consistent response. Further, the presence of two subtypes of FMD in a single patient may suggest the possibility of genetic factors that predispose certain vessels to certain subtypes of FMD.

Fibromuscular Dysplasia May Herald Symptomatic Recurrence of Cervical Artery Dissection

Background: The prevalence of fibromuscular dysplasia (FMD) in patients with cervical artery dissection (CAD) is unknown. Our objectives were to assess the risk of CAD recurring as a stroke or a transient ischemic attack and the association of these events with FMD. Methods: We prospectively included and followed 103 consecutive patients who had been admitted for a CAD. The median follow-up was 4 years (range 4 months to 10 years). The main criteria for inclusion were a mural hematoma demonstrated by cervical magnetic resonance imaging and/or signs suggesting CAD on 2 other investigations. FMD was diagnosed on the so-called string of beads pattern by digital subtraction angiography. Results: Five patients had CAD recurrence (60% occurred late). Four of these 5 patients had FMD. In 4 patients, CAD recurrence involved another cervical artery. Conclusion: The rate of symptomatic CAD recurrence was 1% per year and was often related to FMD.

Renal fibromuscular dysplasia in elderly persons

Renal artery stenosis (RAS) is a significant cause of secondary hypertension, the progression of which can lead to renal insufficiency, uncontrolled hypertension, and even end-stage renal disease. The 2 most common forms of RAS are atherosclerotic renovascular disease and fibromuscular dysplasia (FMD). Atherosclerosis accounts for 90% of all cases of RAS and generally affects an elderly population. Conversely, FMD accounts for approximately 10% of all RAS cases and is described as affecting a younger population. Four cases of FMD in individuals older than 70 years are presented, in a period of 1 year at 1 facility. This case series calls into question the previously reported low prevalence of FMD in elderly persons. It is conceivable that renal artery investigation might be denied an elderly patient thought to have atherosclerotic disease. Because conventional angioplasty is considered the treatment of choice for patients with FMD because of the high response rate for uncontrolled hypertension, the prevalence of FMD in the elderly population should be reevaluated to detect and treat this population accordingly.