Presence Of Fibromuscular Dysplasia Research Articles

“Bead to death,” Fibromuscular Dysplasia Presenting as A Rapidly Enlarging Right Anterior Axillary Mass

Fibromuscular dysplasia (FMD) is a rare non-inflammatory, non-atherosclerotic arterial disease characterized by abnormal cellular proliferation and distorted architecture. It commonly affects the renal and extracranial carotid and vertebral arteries, but nearly all arterial beds may be affected, and multivessel involvement is common. We report a rare case of a 31-year-old Filipino female who presented with a rapidly enlarging right anterior axillary mass. Initial consideration was a bleeding soft tissue sarcoma as seen on chest CT scan, while whole abdominal CT scan with IV contrast revealed incidental findings of multiple aneurysms in the superior mesenteric artery, both renal arteries and right common iliac artery, suggestive of fibromuscular dysplasia. Further vascular imaging studies showed a looped left internal carotid, and a tortuous left brachial artery with beading pattern.

Clinical characteristics and predictors of recurrence from the Australia-New Zealand Spontaneous Coronary Artery Dissection (ANZ-SCAD) registry

Abstract Background Spontaneous coronary artery dissection (SCAD) is an increasingly recognised cause of acute coronary syndrome (ACS), particularly in young women. Our understanding of SCAD remains incomplete with limited prospective data on determinants of SCAD recurrence. Purpose We aimed to describe the clinical characteristics, incidence and predictors of SCAD recurrence and major adverse cardiovascular events (MACE) in patients with SCAD from a large, multicentre registry. Methods Observational, multi-centre prospective and retrospective cohort study recruiting patients with SCAD from 23 hospitals in Australia and New Zealand. Patients aged=>18 years with SCAD and an ACS were eligible for inclusion. Prospectively recruited patients gave their informed consent while a waiver of consent was granted for retrospectively recruited patients. An independent core laboratory adjudicated invasive coronary angiography images to confirm the diagnosis of SCAD. The primary outcome was SCAD recurrence, defined as de novo spontaneous dissection with new ACS symptoms and cardiac enzyme elevation, not due to extension of the index lesion. Cox proportional hazard models were used to evaluate the association between SCAD recurrence and predefined clinical variables. Results From a total 487 patients recruited, 423 (132 prospective and 291 retrospective) patients with SCAD confirmed on core laboratory review were included for analysis. Mean age was 52.4±10.7 years, 89.7% were female and 73.1% were Caucasian. At discharge, 75.2% were on beta-blockers, 27.9% single antiplatelet and 61.9% dual antiplatelet (DAPT). At 15 (interquartile range 5-36) months median follow-up, SCAD recurrence occurred in 3.3%, with an overall MACE of 6.6%. On multivariate analysis, the presence of fibromuscular dysplasia (FMD) or other extra-cardiac vascular abnormalities [adjusted hazard ratio (HR) 5.63, 95% confidence interval (CI), 2.09-15.3, p<0.001], and past history of stroke (adjusted HR 7.99, 95% CI, 1.51-42.4, p=0.015) were associated with higher SCAD recurrence. Discharge beta-blockers was not independently associated with SCAD recurrence, while dual antiplatelet therapy that combined ticagrelor with aspirin was associated with a higher risk of recurrence (adjusted HR 2.47, 95% CI, 1.9-15.6, p=0.048). Conclusion SCAD recurrence comprised half of overall MACE in people with SCAD. The risk of SCAD recurrence was more than 5-fold higher in patients with FMD or other extra-cardiac vascular abnormalities. Dual-antiplatelet therapy that comprised ticagrelor and aspirin was associated with a higher risk of SCAD recurrence.

Registre national des dissections coronaires spontanées : le registre DISCO

AimSpontaneous coronary artery dissection (SCAD) is a form of acute coronary syndrome (ACS). The aim of this registry is to assess the clinical and angiographic features of SCAD, to describe the therapeutic management and prognosis, and to identify links with other vascular diseases. MethodFrom 2016 to 2018, 424 patients with a diagnosis of SCAD were included prospectively and retrospectively in 51 French cardiology centres. Results373 patients with confirmed SCAD were included. The mean age was 51.5±10.3 years with 90.6% women. 54.7% of patients had <2 cardiovascular risk factors. ACS occurred in 96.2% of patients. 84.2% of patients were managed conservatively, 15.5% interventionally and 0.3% surgically.At 1-year follow-up, recurrence of SCAD occurred in 3.3%. No deaths occurred.The association with fibro-muscular dysplasia was found in 45% of cases and genetic analysis confirmed a strong relationship between the occurrence of SCAD and gene variations at the PHACTR1 locus. ConclusionThe DISCO registry is the largest European cohort of SCAD. It confirms that this disease mainly affects young women with few cardiovascular risk factors, and that there is a strong association with the presence of fibromuscular dysplasia (45%). Conservative management should be preferred, with a favourable prognosis (no deaths at 1 year; recurrence rate of 3.3%).

A case of limb shaking transient ischaemic attack due to internal carotid artery dissection: an unusual presentation of fibromuscular dysplasia

BackgroundFibromuscular dysplasia (FMD) has a high prevalence of associated nontraumatic carotid artery dissection, which could further result in transient ischaemic attack (TIA) or stroke. Limb shaking TIA is an unusual form of TIA that is commonly discribed in elderly patients with atherosclerotic backgrounds, while there are limited data about it in patients with FMD. Furthermore, discussions of limb shaking TIA in nonelderly patients are scarce.Case presentationAn Asian 47-year-old female presented with intermittent involuntary movement of the left upper limb accompanied by neck torsion. The episode stopped soon after changing to the supine position. On native source images of time-of-flight magnetic resonance angiography (TOF-MRA), the right internal carotid artery showed a "dual lumen sign" with an intimal flap. On contrast-enhanced magnetic resonance angiography and sagittal black-blood T1WI, an intravascular haematoma with irregular lumen stenosis was observed, which overall indicated right internal carotid artery dissection. Digital subtraction angiography showed the characteristic “string-of-beads” appearance in the left internal carotid artery, and the presence of this sign pointed to the diagnosis of FMD. The patient was finally diagnosed with limb shaking TIA due to internal carotid dissection with fibromuscular dysplasia. The patient was prescribed dual anti-platelet therapy. The limb shaking vanished soon after admission with no reoccurrence in the three-month follow-up.ConclusionsThis case demonstrates that limb shaking TIA can present in patients with FMD. Limb shaking TIA in nonelderly patients can be caused by multiple diseases, and more detailed patient guidance is required in clinical practice.

RENAL EXTRACTION EFFICIENCY FOR PAH IS COMPARABLE IN ESSENTIAL HYPERTENSION AND RENAL FIBROMUSCULAR DYSPLASIA

Objective: Previously, we demonstrated that intrarenal blood flow in most patients with renal fibromuscular dysplasia (FMD) does not differ from that of patients with essential hypertension and patent renal arteries. In the present study, we assessed whether the renal extraction of para-aminohippurate (PAH) as a marker of tubular function was affected by the presence of FMD. Design and method: We studied 136 hypertensive patients who were scheduled for diagnostic renal angiography with arterial and renal venous blood sampling. In all of them, a continuous infusion of PAH was started two hours before the procedure to obtain steady state plasma levels at the time of sampling. Blood was collected simultaneously from the arterial and venous catheter prior to the administration of contrast material. Mean renal blood flow (MRBF) was measured simultaneously using the 133Xenon washout technique. Results: Altogether, 21 patients were diagnosed with renal FMD, 45 with atherosclerotic renal artery stenosis (ARAS) and 70 with essential hypertension (EH). Extraction ratio of PAH (ER-PAH) in the patients with renal FMD averaged 72 + 4% which did not differ significantly from that in patients with EH (74 + 3%). MBRF did not differ between these two groups either. Per contra, in patients with ARAS both MRBF and ER-PAH were significantly lower as compared to the other groups. ER-PAH in FMD patients was not modified by age, sex, height of blood pressure or MBRF. Conclusions: The ER of PAH in patients with renal FMD does not differ from that in EH. Thus, both flow and tubular function are well preserved in FMD when is EH is taken as control. This suggests that the pathophysiological mechanism which contribute to hypertension in FMD are different from those which are active in ARAS.

371 Fibromuscular Dysplasia Presenting as Acute Unilateral Renal Infarction in a Normotensive Patient

371 Fibromuscular Dysplasia Presenting as Acute Unilateral Renal Infarction in a Normotensive Patient

High prevalence of extrarenal artery involvement in children with fibromuscular dysplasia - a single-center experience.

Although the clinical presentation of fibromuscular dysplasia (FMD) and its generalized character has been previously described in adults, data on FMD in children are limited. In this study, we aimed to assess visceral artery involvement in pediatric FMD patients with documented renal artery stenosis (RAS) and renovascular hypertension (RVH) in comparison with healthy individuals. We retrospectively analyzed the results of angiographic studies of 16 patients with a median age of 13.9 years (10 girls) in comparison with 16 age- and sex-matched healthy controls. Out of the 16 FMD patients, 10 (63%) had stenotic lesions identifiable only in renal arteries, whereas six (37%) had additional stenoses in other vascular beds - in the celiac trunk (four patients), superior mesenteric artery (four patients), inferior mesenteric artery (one patient), splenic artery (one patient), common hepatic artery (three patients), and abdominal aorta (one patient). The comparison of ostial diameters of vessels, in which no periostial narrowing, stents, or occlusions were found, revealed that patients with FMD had a significantly smaller diameter of the celiac trunk (P = 0.017), splenic arteries (P = 0.007), and common hepatic artery (P = 0.026) than their age- and sex-matched healthy peers. We found that 69% of children with RVH caused by FMD had clinically silent stenoses or tortuosity of visceral arteries. The results of our study suggest that pediatric FMD is a generalized arterial condition, and the patients may need screening for both renal as well as nonrenal manifestations of the disease.

Unusual presentation of fibromuscular dysplasia presented with hand knob syndrome associated with ipsilateral proximal internal carotid atherosclerotic stenosis

Unusual presentation of fibromuscular dysplasia presented with hand knob syndrome associated with ipsilateral proximal internal carotid atherosclerotic stenosis

Safety Assessment of Endovascular Treatment of Cerebral Aneurysms in Patients with Fibromuscular Dysplasia

Background: The prevalence of cerebral aneurysms is increased in fibromuscular dysplasia (FMD). The presence of FMD may serve as discouragement to elective endovascular aneurysm treatment. Outcomes of endovascular intervention for aneurysms through vessels affected by FMD have not been reported. Methods: A prospectively maintained database of patients undergoing intracranial embolization was reviewed for patients with FMD who underwent endovascular aneurysm treatment. Results: A total of 1,025 patients were screened and 31 (3.0%) had cerebrovascular FMD. These patients underwent a total of 43 embolization procedures; 27 of these procedures were performed through an affected vessel. All but 1 patient were female and the average age was 62 years. “String-of-pearls”-type FMD was the most common subtype (90%). The internal carotid arteries were more commonly affected (65%) than the vertebral arteries (48%). All patients underwent treatment of cerebral aneurysms, most of which (87%) were incidentally discovered; 6 patients (19%) also had incidental vessel dissection. The average aneurysm size was 7.1 mm. The morphology was saccular in 93% of the cases, and 86% were in the anterior circulation. The most commonly performed treatment was flow diversion (67%), in the majority of cases by pipeline embolization. Other procedures performed were coiling (19%), stent-coiling (12%), and intrasaccular flow disruption (2%). All but 1 procedure (98%) were successful. There were no major complications; 1 patient experienced a transient ischemic attack. Follow-up angiography was performed in 88% of the cases, without evidence for disease progression after treatment. The average time to last angiographic follow-up was 17 months (±13). Conclusions: Elective embolization of intracranial aneurysms can be performed safely through vessels affected by FMD.

Fibromuscular Dysplasia Presenting as Spontaneous Coronary Artery Dissection

Fibromuscular Dysplasia Presenting as Spontaneous Coronary Artery Dissection

Association between carotid artery tortuosity and carotid dissection: a case-control study.

Carotid artery dissections have long been associated with compromise of the structural integrity of the arterial wall from heritable connective-tissue disorders, hypertension, and trauma. However, an association between spontaneous internal carotid artery dissection and tortuous or redundant carotid anatomy has not been fully explored. Patients with CTA confirmed spontaneous cervical internal carotid artery dissections were compared to a group of age and sex matched controls who also received CTA of the neck. Patients with trauma or aortic dissections were excluded. Five radiologists reviewed the CTA images to evaluate internal carotid artery tortuosity (reported as loops, kinks or coils), retrojugular and retropharyngeal courses of the internal carotid artery, presence of fibromuscular dysplasia and presence of atherosclerotic disease. Baseline data collected included demographic characteristics (sex, age, smoking history) and cardiovascular comorbidities. A total of 83 cervical internal carotid artery dissection and their age and sex matched controls were included in this study. 46% of patients were female in each group and mean age was 49.2±10.6 years. The presence of any carotid tortuosity was 53% (N.=44) and 34% (N.=28) in the per-patient analysis of dissection and control groups, respectively (P=0.02). Loops were reported in 22% (N.=18) of dissection patients and 8% (N.=7) of controls (P=0.03). Retrojugular course of the internal carotid artery were seen in 23% (N.=38) of dissection patients and 9% (N.=15) of controls (P=0.0009) in the per-vessel analysis. Our study suggests that there is an association between the presence of tortuous carotid artery anatomy and spontaneous carotid artery dissection. This finding emphasizes the importance of the presence of tortuous arteries on CTA imaging to increase the index of suspicion for a potential dissection.

Internal Carotid Artery S-Shaped Curve as a Marker of Fibromuscular Dysplasia in Dissection-Related Acute Ischemic Stroke

Background and Purpose: Craniocervical fibromuscular dysplasia (FMD) is associated with dissections and with S-shaped curves in the internal carotid artery (ICA). We evaluated the occurrence of S-curves in patients presenting with acute strokes due to ICA steno-occlusive dissections. Methods: This was a retrospective review of the interventional databases of two academic tertiary-care institutions. The presence of ICA S-shaped curves, C-shaped curves, 360-degree loops, as well as classic FMD and atherosclerotic changes at the ICA bulb and curve/loop was determined. Cases of carotid dissections were compared with a control group (consecutive non-tandem anterior circulation strokes). Results: Twenty-four patients with carotid dissections were compared to 92 controls. Baseline characteristics and procedural variables were similar, with the exception of younger age, less frequent history of hypertension, diabetes, atrial fibrillation and stent retriever use in patients with dissections. The rates of mTICI2b-3 reperfusion, parenchymal hematoma, good outcome and mortality were similar amongst groups. The frequency of S-curves (any side without superimposed atherosclerosis) was 29% in the dissection group versus 7% in controls (p < 0.01). S-curves were typically mirror images within the dissection group (85% had bilateral occurrence). The frequency of C-shaped and 360-degree curves was similar between groups. FMD changes within the craniocervical arteries were statistically more common in dissection patients. Ten patients (41%) of the dissection group had S-curves or classic FMD changes. Multivariate analysis indicated that S-curves were independently associated with the presence of dissections. Conclusion: S-shaped ICA curves are predictably bilateral, highly associated with carotid dissections in patients with moderate to severe strokes, and may suggest an underlying presence of FMD.

Abstract TP18: S-shaped Internal Carotid Artery Curve in Dissection-related Acute Ischemic Stroke

Background and Purpose: Craniocervical fibromuscular dysplasia (FMD) has been associated with dissections. Recently, the presence of an S-shaped curve in the ICA has been described as a marker of FMD. We evaluated the occurrence of S-shaped curves in acute ischemic stroke (AIS) patients presenting with ICA steno-occlusive dissections. Methods: This was a retrospective review of the interventional database of two academic tertiary care institutions. Cases of carotid dissections were compared with a control group (consecutive non-tandem anterior circulation strokes). The presence of ICA S-shaped curves, C-shaped curves, 360° loops, as well as, FMD changes and atherosclerotic changes at the ICA bulb and curve/loop was determined by evaluation of conventional-angiogram and CTA. Results: Twenty-four patients with carotid dissections were identified and compared to 92 controls. The baseline characteristics were similar, with the exception of expected younger age and less prevalent history of hypertension, diabetes and atrial fibrillation in the dissection group. Procedural variables were also similar, with the exception of a large percentage of the use of stentretrievers in the control group. The occurrence of parenchymal hemorrhages, good outcomes and mortality were similar amongst the two groups. The frequency of S-shaped curves on either side was 29% in the dissection group vs. 7% in the control group (p&lt;0.01). The S-shaped ICA curves were typically mirror images within the dissection group (85% of the patients with an S-shaped curves had bilaterally occurrence). The frequency of C-shaped and 360 degree curves was statistically similar between groups. FMD changes within the craniocervical arteries were statistically more common in the patients with dissections. Ten patients of the dissection group had either FMD changes or an S-Shaped curve (41%). Multivariate analysis indicated that S-shaped curves were independently associated with the presence of dissections. Conclusion: S-shaped ICA curves are predictably bilateral, independently associated with carotid dissections in patients undergoing thrombectomy for acute ischemic stroke, and may indicate the underlying presence of FMD.

Abstract 15492: Spontaneous Coronary Artery Dissection: Characterizing Presentation, Management, and Outcomes at a Large Referral Cardiovascular Center

Introduction: Non-atherosclerotic spontaneous coronary artery dissection (SCAD) primarily affects women and may be associated with arteriopathies such as fibromuscular dysplasia (FMD). We aimed to describe patient characteristics, management, and outcome of SCAD at a large referral center. Methods: We queried our institutional cardiac cath database for SCAD diagnoses from 2000-2014. The electronic medical record was also queried for consecutive SCAD patients seen in a specialty arteriopathy clinic from 2010-2015. Details regarding presentation, treatment, and outcome of SCAD were collected. Trends in management over two time periods (2000-2009, 2010-2015) were compared. Results: There were 96 SCAD events in 75 patients (16 patients had one recurrence, one had three events, and one had four distinct events). Mean age at presentation was 47 years, 83% were female, and 84% presented with ACS. The LAD was the most commonly affected vessel (54%). Most SCAD events were treated medically (52%). Of 41 events treated with PCI, 19 were complicated by hematoma extension. Four PCI patients required subsequent CABG. Imaging of other arterial beds increased over time, with 2/3 patients receiving vascular ultrasound to evaluate for renal or carotid FMD in the more recent time period (Table). Of those evaluated for FMD, 45% had imaging findings compatible with multifocal FMD. Significant carotid tortuosity was present in 38%, with four demonstrating a classic “S surve” appearance recently shown to be a variant presentation of FMD. All patients survived to index hospitalization discharge, however, 24% of patients had at least one recurrence. Conclusions: SCAD affects predominantly young females and often manifests as ACS. Findings of FMD or carotid tortuosity are frequent and recognition of SCAD as a potential manifestation of FMD is increasing in our institution. In support of other reports, recurrence of SCAD is not rare and PCI attempts are complicated nearly half of the time.

Pathologie du médiastin. Cas no 2. Lymphome B inclassable, avec des caractéristiques intermédiaires entre un lymphome de Hodgkin classique et un lymphome B à grandes cellules primitif du médiastin

We present a unique case of sudden death in a 21-year-old man with history of cocaine use and a solitary fibromuscular dysplastic lesion completely occluding the left coronary artery ostium. We document intimal proliferation of myofibroblasts at the opening of the left coronary ostium without other concomitant lesions. This report discusses the gross and histologic features of the lesion, explores in careful detail the possible etiologies, and gives a comprehensive literature review of isolated coronary ostial fibromuscular dysplasia presenting with sudden death.

Prevalence of Extracoronary Vascular Abnormalities and Fibromuscular Dysplasia in Patients With Spontaneous Coronary Artery Dissection

Spontaneous coronary artery dissection (SCAD) is a cause of acute coronary syndrome, often occurring in young women. The utility of comprehensive imaging and clinical significance of detected vascular abnormalities have yet to be determined. We hypothesized that extracoronary vascular abnormalities (EVAs) are common in SCAD and aimed to study the prevalence and distribution of these findings. We enrolled 115 patients with confirmed SCAD who were evaluated at the Mayo Clinic SCAD Clinic from February 2010 to May 2014 and prospectively underwent comprehensive computed tomography angiography imaging of the neck, chest, abdomen, and pelvis (SCAD computed tomography angiography protocol, n=95) or had retrospective review of outside studies (n= 20) including head imaging (n=40). Follow-up was determined by last clinical visit or study correspondence and included review of recurrent SCAD or myocardial infarction, congestive heart failure, and death. We reported EVAs in 66% of patients with SCAD, most frequently in the abdomen (36%), pelvis (28%), and neck (27%). Only 1 patient had EVA in the chest (aortic dissection and Marfan's). Fibromuscular dysplasia (FMD) (exclusively multifocal) was the most common type of EVA (45%). Vascular abnormalities in those with head imaging included intracranial aneurysms (n = 9) and FMD (n = 3). There were no deaths at median follow-up of 21months (Q1 to Q3 7.7 to 55). The presence of FMD was not associated with SCAD recurrence (relative risk [RR] 1.2; confidence interval [95% CI] 0.60, 2.5), congestive heart failure (RR 0.66; 95% CI 0.20, 2.3), or myocardial infarction (RR 1.34; 95% CI 0.69, 2.6). In conclusion, EVAs including FMD, dissections, aneurysms, and dilation are common in patients with SCAD and occur in a wide anatomic distribution. The presence of EVAs and/or FMD did not correlate with the risk of subsequent clinical events, but future studies with increased power and longer follow-up will be important to further assess the role of EVAs in patients with SCAD.

Endovascular thrombectomy and stenting in the management of carotid fibromuscular dysplasia presenting with major ischemic stroke

Fibromuscular dysplasia (FMD) affecting the internal carotid artery (ICA) is considered a benign condition; however, retinal and cerebral ischemic events may occur. We present a patient with carotid FMD presenting with a major ischemic stroke due to major embolic occlusion of the ICA in conjunction with a hemodynamic component caused by narrowing and beading of the lumen associated with FMD. The patient was successfully treated with intracranial stent-assisted thrombectomy followed by cervical ICA stenting that aimed to reconstruct and angioplasty the FMD-affected arterial segment. Recently, stent-based thrombectomy has emerged as the most effective endovascular option for the rapid revascularization of major intracranial occlusions; however, to our knowledge, its use in a rare case of FMD-associated major stroke has not yet been reported.

Intracranial fibromuscular dysplasia presenting as various ocular manifestations

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that mainly affects renal, extracranial carotid, and vertebral arteries. Intracranial FMD is uncommon unlike extracranial or renal FMD, and the primary manifestation of intracranial FMD is intracranial aneurysm. We report an unusual case of intracranial FMD showing various ocular manifestations, including central retinal artery occlusion, transient monocular blindness, and oculomotor nerve palsy without renal involvement.

Bilateral renal infarction: an uncommon presentation of fibromuscular dysplasia.

While fibromuscular dysplasia (FMD) is an established cause of secondary hypertension, its association with renal infarction is less well recognized. We report a middle-aged man who presented with complaints of loin pain and severe hypertension. Computed tomography angiography of the abdomen revealed bilateral renal infarction with multiple short-segment arterial dissection compatible with FMD in the absence of systemic vasculitis and other risk factors for thromboembolic events. Bilateral renal infarction complicating FMD is extremely rare and has so far been reported only in a handful of cases. Physicians encountering cases of otherwise unexplained renal infarction/ischemia need to be aware of this complication.

Fibromuscular dysplasia presenting with asymptomatic bilateral renal infarctions

Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic vascular disease. It is the second cause of renovascular hypertension after atherosclerosis. Although FMD usually has a good prognosis, renal infarctions and artery dissections have been described. We present the case of a 38-year-old woman with hypertension and asymptomatic bilateral renal infarctions. Bilateral FMD of segmental branches of the renal arteries was diagnosed by digital subtraction angiography after an exhaustive study. Previous intake of nonsteroidal anti-inflammatory drugs may also have played a significant role in the development of renal infarctions. To our knowledge, bilateral renal infarctions complicating FMD have been reported in only four previous cases; only in one of those cases, renal infarctions were asymptomatic.