Diagnosis Of Fibromuscular Dysplasia Research Articles

Abstract P439: An Uncommon and Missed Diagnosis of Hypertension: Fibromuscular Dysplasia

Introduction: Fibromuscular dysplasia is a noninflammatory vascular disease most commonly affecting carotid and renal arteries, especially in young to middle aged women. Renal artery stenosis may cause refractory and severe hypertension in these patients without traditional risk factors. Case: A 58-year-old woman with uncontrolled hypertension on calcium channel and angiotensin receptor blocker and history of left carotid artery stenosis, and carotid endarterectomy at the age of 48 years, was referred cardiac evaluation. Patient’s twin sister had a diagnosis of fibromuscular dysplasia (FMD). Her blood pressure was 190/90 mmHg at presentation. Computed Tomography Angiography (CTA) of the abdomen/pelvis showed a typical nodular stenosis of the mid renal artery consistent with FMD. Patient underwent renal angiogram and successful percutaneous transluminal angioplasty. She still required medical therapy but her BP significantly improved. Discussion: Our patient had severe hypertension, carotid artery stenosis and even surgery but had missed FMD diagnosis for > 10 years. Physicians should have a high level of clinical suspicion for young and middle aged women with refractory hypertension and/or artery stenosis, aneurysm, or dissection in any vascular bed. CTA should be performed for renal and carotid artery imaging looking for typical “string of beads” appearance. Cerebral artery aneurysm should also be evaluated. The inheritance pattern for FMD such as PHACTR1 is most consistent with an autosomal dominant trait with variable penetrance. Renal artery angioplasty may lessen the degree of refractory or severe hypertension.

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.

Abstract 2125: Aneurysmal Disease Among Hospitalized Patients With Fibromuscular Dysplasia - Types And Prevalence: Findings From The National Inpatient Sample

Introduction: Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory disease that can lead to various arterial abnormalities, including dissection, occlusion, and aneurysm. Only a few studies have examined the common types of aneurysmal disease among patients with FMD. We examine the types and prevalence of aneurysmal disease among hospitalized patients with FMD. Methods: Using the International Classification of Diseases, 10th edition, Clinical Modification (ICD-10-CM) diagnosis codes, we queried the National Inpatient Sample 2016 to 2020 for hospitalizations among patients ≥18 years old with a diagnosis of Fibromuscular dysplasia. Aneurysmal disease of the iliac, carotid, upper extremity, lower extremity, renal, vertebral artery, thoracic aorta, and abdominal aorta was further identified in this cohort of patients using ICD-10 codes. We examined prevalence of FMD and further compared the prevalence of aneurysmal disease among hospitalized patients with and without FMD. Results: Our study included 9,315 weighted hospitalizations among patients with fibromuscular dysplasia (mean [SD] age 61.2 [15.5] years). FMD was five times more common in females than males (84.1% vs. 15.9%). Compared to hospitalized patients without FMD, aneurysmal disease was more common among hospitalized patients with FMD. The most common aneurysmal site among patients with and without FMD was the renal artery (1.4%) and abdominal aorta (0.71%) respectively. The prevalence of aneurysmal disease among hospitalized patients with FMD was renal artery aneurysm (1.40%), abdominal aortic aneurysm (1.29%), carotid artery aneurysm (1.23%), thoracic artery aneurysm (1.02%), vertebral artery aneurysm (0.86%), lower extremity artery aneurysm (0.38%), iliac artery aneurysm (0.27%), and upper extremity artery aneurysm (0.16%). Conclusion: Aneurysmal disease is prevalent among hospitalized patients with Fibromuscular dysplasia, with the renal artery identified as the most common site of aneurysm. Further research is needed to understand better the relationship between FMD and other vascular abnormalities.

1279 Do Carotid Webs Raise the Risk of Ischaemic Stroke? a 12 Year Single Centre Retrospective Study

Abstract Aim Carotid webs are a rare type of fibromuscular dysplasia (FMD), thought to be a cause of ischaemic stroke. We aimed to review the presentation and outcomes of patients with carotid webs attending our unit over a 12-year period. Method The cohort of patients with carotid webs who presented to our unit between 2010 and 2021 were identified by electronically interrelating imaging reports (CT/MR/angiogram) for the phase “carotid web”. Images were re-reviewed to confirm the diagnosis. The electronic records for the cohort were also reviewed. Results 60 patients with carotid webs were identified, with an age range of 36 to 92 years (mean age 64 years) and a similar sex distribution (31 male, 29 female). 29 carotid webs were left-sided, 22 were right-sided and 9 were bilateral. None had a pre-event diagnosis of FMD. 27 patients had an ischaemic stroke - 18 in the territory relevant to the web location, 9 in other territories. 1 patient was treated with thrombolysis, 1 with thrombectomy and 1 with thrombolysis and thrombectomy. None underwent carotid intervention. Of the 18 patients with a carotid web on the ipsilateral side to their ischaemic event, none had a repeat event recorded. Conclusions 30% of the cohort had an ischaemic stroke event corresponding to the territory supplied by a carotid artery with a web. Current management of carotid webs leans towards conservative management with antiplatelet therapy. However, these data suggest carotid intervention should be considered in selected cases where stroke symptoms correlate to the laterality of the carotid web.

Stroke Secondary to Fibromuscular Dysplasia

FMD is a rare systemic vascular disease characterized by abnormal cell proliferation in the artery walls, leading to artery narrowing, twisting, or bulging. It commonly affects the carotid and renal arteries and can result in renal diseases and stroke. This paper presents a case study of a 39-year-old unhoused female patient who experienced a stroke secondary to fibromuscular dysplasia (FMD) and left ventricular thrombus. The patient presented with asymmetric movement of the extremities and psychosis, so intoxication was initially suspected. However, further examination revealed facial asymmetry and motor weakness. CT scans showed a left anterior cerebral artery infarction and irregular contour of the cervical internal carotid arteries, consistent with FMD and carotid thrombus. Management involved stabilizing the patient, providing supportive treatment, and controlling blood pressure. Thrombolytic therapy was not administered due to the time elapsed since symptom onset. The patient was started on anticoagulation for the carotid plaque and left ventricular thrombus. A transthoracic echocardiogram revealed a large left ventricular thrombus and left atrial enlargement, and the patient was started on heart failure medications. FMD and left ventricular thrombus are not directly related, but FMD can contribute to thrombus development through its impact on the cardiovascular system, including hypertension, arterial wall damage, aneurysm development, or dissection. Treatment for FMD may involve medication, observation, or surgical interventions like angioplasty and stenting. Anticoagulation therapy is essential for managing the left ventricular thrombus. This case highlights the importance of early diagnosis and treatment of FMD to prevent complications such as stroke. It is important to consider FMD in the diagnosis of younger patients with a stroke, especially those with a history of hypertension, substance abuse, or other risk factors. Further research is needed to better understand the relationship between FMD and stroke and to optimize treatment strategies for these patients.

PREVALENCE AND DISEASE SPECTRUM OF FIBROMUSCULAR DYSPLASIA IN PATIENTS WITH SPONTANEOUS CEREBROVASCULAR ARTERY DISSECTION

Objective: Spontaneous cervical artery dissection (sCeAD) is the most frequent cause of ischemic stroke in young adults and has been associated to various arteriopathies, such as fibromuscular dysplasia (FMD). The aim of this study was to assess the prevalence of both cervical and extra-cervical FMD in patients with a diagnosis of CeAD. Design and method: All patients discharged from the Stroke Unit of A.O.U. Città della Salute e della Scienza of Turin from 2016 to 2020 with a diagnosis of CeAD underwent a contrast-enhanced CTA from chest to pelvis to look for FMD-related lesions (multifocal stenosis, aneurysms, dissections, arterial tortuosity). FMD diagnosis was assessed in the presence of at least a multifocal stenosis in one of the vascular beds explored. Results: Among 56 patients with spontaneous CeAD, 72.7% were male of middle age (52±8.5 y.o.). According to radiological findings, 43.2% of patients showed extra-cervical arterial lesions, with a prevalence of overall FMD (defined as the presence of at least a multifocal stenosis) reaching 47.3% of the studied sample and affecting carotid (36.4%), renal (18.9%), visceral (16.2%), iliac (22.2%) and coronary arteries (2.7%) respectively. Renal involvement included multifocal renal stenosis (13.5%) and arterial aneurysms (5.4%). Visceral involvement included multifocal stenosis (13.5%), visceral aneurysms (2.7%) and silent mesenteric artery dissection (5.4%). Iliac involvement included multifocal stenosis (2.7%), silent artery dissection (2.7%) and tortuosity (16.7%). Furthermore, two spontaneous dissections, one of the left external iliac artery and the other of the coeliac trunk, clinically asymptomatic, were reported in two males with no multifocal stenosis. Two women suffered also from SCAD. Multivascular extra-cervical involvement was observed in almost one third of the patients (27.0%) with sever lesions (multifocal high degree stenosis and/or arterial dissections) affecting 13.5% of the population. Conclusions: According to our results, we demonstrated that FMD is frequently associated to CeAD and that silent FMD-related lesions, even severe as arterial dissections, may coexist in such patients. Therefore, FMD should always be taken in consideration in the differential diagnosis of spontaneous arterial dissection, especially in young subjects without major cardiovascular risk factors.

Female with Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disorder that cause arterials steno- sis, occlusion, aneurysm, dissection, and artery tortuosity. The etiology of FMD remains unknown. The most frequently involved arteries are the renal, internal carotid, vertebral arteries and visceral. Among adults, FMD is more common in females. Most common symptoms are renovascular hypertension, headache, pulsatile tinnitus, neck pain, and abdominal pain. We present a 62-year old female patient who was diagnosed with grade 3 arterial hypertension at the age of twenty -nine. During diagnostic treatment, narrowing of the right renal artery was determined and percutaneous transcutaneous angioplasty of the right renal artery was performed. Diagnosis of fibromuscular dysplasia was suspected. After this procedure, the value of blood pressure was normalized. At the age of forty, the patient was re-introduced with antihypertensive, and CT angiography showed 50 % lumen stenosis of the right renal artery. She was referred to a neurologist. The patient has been complaining about frequent headaches, pulsatile tinnitus in the right ear, increased fatigue, and occasional abdominal pain for many years. Neurosonological testing showed partially wall thickening in the left common carotid artery and multiple stenosis and dilatation of the left internal carotid artery. The left vertebral artery has multiple stenoses and dilatations in the distal V1 segment. The findings indicated changes in fibromuscular dysplasia and the diagnosis of FMD was confirmed.

JS-NG-3: UNIQUE IMAGING FINDINGS IN RENAL ARTERY FIBROMUSCULAR DYSPLASIA

A 35-year-old female patient with fluctuating blood pressure was referred to hypertension department for investigation of possible secondary hypertension. Computed tomographic angiography (CTA) of the renal arteries was performed, demonstrating abnormal right renal artery at the medial portion of the vessel with complete tubular narrowing and right kidney atrophy (right 79*35 mm, left 109*45 mm) (Panel A). Subsequent renal arteriography confirmed the long tubular narrowed right renal artery (Panel C) and the established severe tortuosity collateral vessel from the abdominal aorta (Panel B). The etiological diagnosis of the renal artery stenosis in this young woman was challenging and was supposed to be congenital renal dysplasia. Catheter-based angioplasty was not recommended. However, intravascular ultrasound (IVUS) revealed that it was not congenital narrowed artery, but concentric intimal-medial thickening caused stenosis of the vessel (Panel E). Therefore, renal artery fibromuscular dysplasia (FMD) was diagnosed and considered to be intimal type. Balloon angioplasty was performed, and IVUS showed that the lesion lumen was dilated successfully (Panel F). However, a bailout stent had to be placed because of intimal dissection during angioplasty, and renal blood flow was restored after procedure (Panel D). One month after the procedure, the patient was normotensive without any antihypertensive medication. At 1 year follow-up, the 99mTc-glomerular filtration rate of the right kidney increased from 19.7 to 40.4 ml/min. FMD is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects renal arteries and is recommended to be classified according to angiographic finding as multifocal FMD or focal FMD. Compared with multifocal FMD, focal FMD is less often seen, and the diagnosis of some cases is challenging. In the present case, such a long tubular stenosis of renal artery is rare, and the diagnosis was confirmed by IVUS. IVUS may be a useful tool for the diagnosis of focal FMD.

JS-FMD: FIBROMUSCULAR DYSPLASIA IN ASIA: A DISEASE TO (RE)DISCOVER

The International Consensus on Fibromuscular Dysplasia (FMD) (Gornik, Persu et al. J Hypertens. 2019;37:229–252; Vasc Med. 2019;24:164–189) provided a comprehensive summary of the clinical history, diagnosis and management of FMD. However, the authors admitted that most of current knowledge on FMD has been acquired in patients of Caucasian descent. Accordingly, they expressed their commitment to include a higher proportion of patients of non-white ethnicities in existing registries. Along these lines, the European International FMD Registry and Initiative (FEIRI, coord. A. Persu, Brussels) has established collaborations with centres from Japan and China and begun to enrol patients from these countries. The 2022 meeting of the International Society of Hypertension in Kyoto is a unique opportunity to further develop the Japanese FMD initiative (coord. Kan Zen, Kyoto), the Chinese FMD initiative (coord. Jiguang Wang, Shanghai) and potentially initiatives in other Asian countries. This implies also raising awareness of Asian experts on FMD and identifying the specificities of FMD in Asia. The objectives of the current session are: (1) to summarize the key messages of the international FMD Consensus; (2) to present preliminary data obtained in patients from Asian vs. Caucasian descent enrolled in the FEIRI registry; (3) to present current experience on the characteristics, diagnosis and management of FMD in centres from Japan (Kyoto, Osaka and Tokyo) and China (Shanghai); (4) to emphasize the specific challenges raised by FMD in Asia, particularly the differential diagnosis with Takayasu disease; (5) to open a debate with specialists of FMD in Japan and abroad, to expand the Japanese FMD initiative and to initiate new national initiatives on FMD in Asia, in collaboration with FEIRI.

Abstract 13419: Racial Differences 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. The demographics and clinical differences based on race in patients with SCAD are not well understood. Methods: Demographics and clinical variables were obtained from the prospective, multicenter iSCAD Registry. Characteristics of study participants (including demographics, past medical history, timeline, imaging studies, follow-up visits, SCAD presentation, and treatment) were compared between white and non-white patients. Race and ethnicity was self-reported by a patient questionnaire. Results: A total of 505 patients were included in the analysis. Among them, 427 (84.6%) were characterized as white and 78 (15.4%) were characterized as non-white. White patients were 49.5 years and non-white patients were 46.8 years at the time of the initial SCAD presentation (p=0.0435). White patients were more likely to be married (77% vs 51%, p<0.0001) and were more likely to have a college degree (42 vs 28%, p<0.0001). White patients were more likely to present to the hospital in less than 24 hours (85% vs 75%, p=0.07) and have an angiogram in less than 24 hours (78% vs 58%, p=0.004). White patients were more likely to present with STEMI (30% vs 11%, p=0.004) and were more likely to receive revascularization (25% vs 11%, p=0.0147). A multivariable analysis explored the variables associated with angiography greater than 24 hours. STEMI vs NSTEMI [OR 0.090 (0.035 to 0.231), p<0.0001] and white vs non-white [OR 0.376 (0.188 to 0.755), p=0.0059] remained significant. White patients were more likely to have fibromuscular dysplasia (FMD), (43% vs 30%, p=0.0336). There was no significant difference in the number of non-coronary vascular imaging studies between the two groups. Conclusions: The number of non-white patients enrolled in the iSCAD registry is low. White patients with SCAD were more likely to present with STEMI and were more likely to have a diagnosis of FMD. Further research is needed to study associations between race and differences in presentation, comorbidities, and treatment in patients with SCAD.

Abstract 13836: Factors Associated With a Complicated Hospital Course in Patients With Spontaneous Coronary Artery Dissection: A Report of the iSCAD Registry

Introduction: Spontaneous coronary artery dissection (SCAD) is a cause of acute coronary syndrome in younger patients. Though associated with lower short and long-term mortality, there is still a need to identify the subset of patients who are at a higher risk of adverse events or a more complex hospital course. Methods: Using data from the iSCAD Registry, a multicenter registry of patients with SCAD, univariate and multivariable logistic regression models were created to assess the association between factors related to the index admission and a complicated hospital course. A complicated hospital course was defined as the occurrence of either a recurrent myocardial infarction (MI), cerebrovascular accident (CVA), a new arrhythmia, heart failure requiring diuretics, ≥ 2 angiograms performed, or a hospital admission of more than 5 days. The multivariable logistic regression model was developed using a backward selection approach with exit criteria set at p >0.2. Patients presenting with cardiac arrest were excluded. Results: Of the 414 patients included, 159 patients (38.4%) had a complicated hospital course: 76 patients had a prolonged admission, 27 had a recurrent MI, 1 had a CVA, 39 had an arrhythmia, 13 had heart failure requiring diuretics and 72 had ≥ 2 angiograms in the index admission. The final model showed that patients who had structural complications related to MI, fibromuscular dysplasia (FMD), or a history of cardiomyopathy were more likely to experience a complicated hospital course (Table). Finally, patients on anticoagulation were at a higher risk of having the composite outcome but this may be secondary to the indication for anticoagulation. Conclusions: The presence of structural complications related to MI, a diagnosis of FMD, or a history of cardiomyopathy were associated with an increased risk of a complicated hospital course related to SCAD. Identification of risk factors for a complex inpatient stay may help to tailor the acute care of SCAD patients.

Aneurysms of the superior mesenteric artery and its branches

ObjectiveAneurysms of the superior mesenteric artery (SMA) and its branches are rare and account for only 6% to 15% of all visceral artery aneurysms. In the present report, we have described our 30-year experience with the management of aneurysms of the SMA and its branches at a high-volume referral center. MethodsA retrospective review of all patients with a diagnosis of an aneurysm of the SMA or one of its branches from 1988 to 2018 was performed. Pseudoaneurysms and mycotic aneurysms were excluded. The clinical presentation, etiology, aneurysm shape and size, treatment modalities, and outcomes were analyzed. The growth rate of the aneurysms was estimated using linear regression. ResultsA total of 131 patients with 144 aneurysms were reviewed. The patients were primarily men (64%), with a median age of 60 years. Of the 144 aneurysms, 57 were fusiform, 30 were saccular, and 57 were dissection-associated aneurysms. Of the 131 patients, 41 had had an isolated SMA branch aneurysm. Degenerative aneurysms were the most common etiology (66%). A total of 35 patients (27%) were symptomatic at presentation. Of the 144 aneurysms, 111 had multiple computed tomography angiograms available, with a median follow-up of 43.6 months (interquartile range, 10.6-87.2 months). Only 18 aneurysms (16%) had had an estimated growth rate of ≥1.0 mm/y. The initial aneurysm size was significantly associated with the growth rate for the fusiform aneurysms (odds ratio [OR], 1.13; 95% confidence interval [CI], 1.0-1.3]; P = .02) but not for the saccular (OR, 0.91; 95% CI, 0.76-1.1; P = 1.1) or dissection-associated (OR, 1.2; 95% CI, 0.91-1.5; P = .20) aneurysms. Acute abdominal pain (OR, 5.9; 95% CI, 1.6-22; P = .01) and chronic abdominal pain (OR, 3.7; 95% CI, 1.1-13; P = .04) were associated with aneurysm growth. Only two patients had a ruptured aneurysm, both of whom presented with rupture with no prior imaging studies. These two patients had a diagnosis of fibromuscular dysplasia and systemic lupus erythematosus, respectively. Of the 131 patients, 46 (34%) had undergone operative repair, including 36 open revascularizations and 8 endovascular procedures. The average aneurysm size for these 46 patients was 24.0 ± 8.6 mm. One patient died perioperatively, and nine patients experienced perioperative complications (25%). Of the 144 aneurysms, 91 were <20 mm, with an average size of 13.4 ± 3.1 mm. These 91 aneurysms had been followed up for a median of 120.8 months (interquartile range, 30.5-232.2 months), with no ruptures within this cohort during the follow-up period. ConclusionsThe present study represents one of the largest series on aneurysms of the SMA and its branches. Our results showed that aneurysms of the SMA are relatively stable. Patients with symptomatic and fusiform aneurysms had a greater risk of growth. Aneurysms <20 mm with a degenerative etiology can be safely monitored without treatment.

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.

1487 The Pied-Piper, How Gastroenteritis Led Us Astray. Fibromuscular Dysplasia: A Surgical Perspective of a Rare Vascular Disorder

Abstract A 73-year-old female was admitted for abdominal pain after eating an out-of-date pie. She was treated for gastroenteritis for 48hours, after which she became unexpectedly haemodynamically unstable. Computerised Tomography (CT) suggested mesocolic arterial bleed with a large mesenteric haematoma. CT angiography confirmed false aneurysms of the middle colic artery and multiple beading of coeliac branches, small mesenteric vessels and the inferior mesenteric artery. Opinion was sought from multiple specialties, pseudoaneurysm embolisation was undertaken and steroids commenced for presumed vasculitis. Vasculitis screen was negative, and PET CT scan showed no metabolically active vasculitis. It was felt a diagnosis of fibromuscular dysplasia (FMD) was likely. Steroids have been weaned and the patient is under active observation. Background FMD is a rare idiopathic condition of abnormal cellular growth in medium/large arterial vessel walls. It has a frequency of approximately 0.02%, predominantly affecting middle-aged Caucasian women. It manifests mostly in renal and cerebrovascular arteries and can present as hypertension or stroke. Complications include aneurysms, stenosis and dissections. Characteristic angiographical finding is a “beads on a string” appearance. There is no current cure, but surgical or interventional involvement by angioplasty and stenting may play a role treatment. Conclusions This case elegantly highlights the ambiguity in diagnosis of abdominal pain in a general surgical setting; with a wide variety of differentials spanning multiple specialties, it emphasises the importance to consider rare presentations of equally rare pathologies. It is a celebration of the merits of a multi-disciplinary approach to solve complex clinical questions.

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021.

Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.

Role of Digital Subtraction Angiography in Diagnosis of Fibromusculardysplasia

Fibromuscular dysplasia (FMD) is a vascular disease characterized by abnormal arterial wall architecture, usually visualized angiographically as the classic “string of beads” sign. We present the diagnostic dilemma of a 51-year-old woman admitted for a spontaneous renal infarct, with initial examination and investigations equivocal. She was consequently assessed for thromboembolic and vasculitic disease, before proceeding to digital subtraction angiography (DSA). This invasive method elucidated an area of focal FMD, previously not seen on computed tomography angiography (CTA). This is unusual due to CTA's high sensitivity and specificity but could be attributed to the less common subtype of FMD which requires combined imaging modalities to arrive at a diagnosis. Hence, there may be a role for the dual use of CTA and DSA in young patients presenting with a spontaneous renal infarct in the absence of other diagnoses.

Male Sex Is Associated With Cervical Artery Dissection in Patients With Fibromuscular Dysplasia.

BackgroundCervical artery dissection (CeAD) is a frequent manifestation of fibromuscular dysplasia (FMD). However, risk factors for CeAD are unknown. We investigated factors associated with CeAD in the ARCADIA (Assessment of Renal and Cervical Artery Dysplasia) registry.Methods and ResultsThe ARCADIA registry includes women or men aged ≥18 years, with a diagnosis of renal, cervical, or intracranial artery FMD, who were prospectively recruited at 16 university hospitals in France and Belgium. Diagnosis of acute or past CeAD at inclusion was established on imaging according to standard diagnostic criteria. Associations between potential determinants and CeAD were assessed by logistic regression analyses. Among 469 patients (75 men) with FMD, 65 (13.9%) had CeAD. Patients with CeAD were younger, more likely to be men, have a history of migraine, and less likely to have a history of hypertension than patients without CeAD. In the multivariable analysis, male sex (odds ratio [OR], 2.66; 95% CI, 1.34–5.25), history of migraine (OR, 1.90; 95% CI, 1.06–3.39), age ≥50 years (OR, 0.41; 95% CI, 0.23–0.73), history of hypertension (OR, 0.35; 95% CI, 0.20–0.64), and involvement of ≥3 vascular beds (OR, 2.49; 95% CI, 1.15–5.40) were significantly associated with CeAD. To validate the association between CeAD and sex, we performed a systematic review. We collected additional data on sex from 2 published studies and unpublished data from the US Registry for Fibromuscular Dysplasia and the European/International FMD Registry. In the pooled analysis (289 CeAD, 1933 patients), male sex was significantly associated with CeAD (OR, 2.04; 95% CI, 1.41–2.95; I 2=0%).ConclusionsIn patients with FMD, male sex and multisite involvement are associated with CeAD, in addition to other previously known risk factors.RegistrationURL: https://www.clinicaltrials.gov; Unique identifier: NCT02884141.

A SNAPSHOT FROM AN EASTERN EUROPEAN REGISTRY ON FIBROMUSCULAR DYSPLASIA

Objective: To evaluate the specific characteristics of patients with fibromuscular dysplasia (FMD) with various localizations and clinical manifestations (or without). The diagnosis is made with angiography. Design and method: This is a case series of 27 consecutive patients with FMD, who were diagnosed in the Cardiology clinic of UMHAT “Alexandrovska” for a period oof 4 years. The diagnosis is based on angiographic criteria, but in several cases an intravascular ultrasound (IVUS) was needed for confirmation, because of discrepancies with the universal understanding for the angiographic image and localization of lesions. Results: The mean age at the time of diagnosis was about 58 years; males were 6 (22%). Renal localization had 17 (63%); extracranial localization – 7 (26%); intracranial – 2 (7.4%); other (3.7%). The age at diagnosis was relatively high. Nine (33%) of the patients underwent interventional treatment in accordance with the contemporary guidelines. Both groups intervened/non-intervened should be routinely followed (at least with ultrasound) in order to properly assess their cardiovascular risk. Conclusions: All the cases were scheduled for angiography for a variety of symptoms: neurological, cardio-vascular, or for embolization of a renal cell carcinoma. Although there are clear criteria for the diagnosis of FMD, in several cases an IVUS was needed for confirmation and exclusion of the differential diagnoses. In the future, clear IVUS criteria may be reasonable to be included in the diagnostic process, especially in cases with borderline angiographic morphology or in those without any typical clinical manifestation.

Abstract 13231: Fibromuscular Dysplasia Screening in Patients With Spontaneous Coronary Artery Dissection

Introduction: Spontaneous coronary artery dissection (SCAD) is commonly associated with fibromuscular dysplasia (FMD), a vascular condition that can lead to aneurysm and dissection in any arterial bed. SCAD and FMD are increasingly being diagnosed, especially in women. Our objective is to describe screening patterns of FMD over time among patients with SCAD. Methods: We conducted a retrospective cohort study, identifying patients treated for SCAD in the University of Pennsylvania Health System from 2005-2019 using ICD-9 (414.12) and ICD-10 (I25.42) codes. Charts were manually reviewed and clinical data were abstracted. Screening modalities included CT angiogram (CTA) or MR angiogram (MRA) of the head, neck, or abdomen; carotid or renovascular ultrasound; or renal angiography. Complete screening was defined as having head, neck, and abdominal imaging. We indexed cases by date of SCAD event. Time categories were calculated by quartiles of patients. We used Fisher’s exact test to compare proportions and Cochran-Armitage trend test to compare trends over time. Results: We identified 121 patients with SCAD with ≥ 6 months of follow-up prior to the COVID-19 pandemic. FMD screening rates by any modality were higher among patients with SCAD in 2018-2019 compared to 1993-2011 (62% vs. 28%, p=0.009 for trend), see Figure. Rates of complete screening remained low and did not change over time (p=0.451 for trend). In 2018-2019, only 41% of patients were screened with CTA or MRA of the abdomen, 26% with CTA or MRA of the neck, and 15% with CTA or MRA of the head. Patients undergoing complete screening had higher rates of FMD diagnosis compared to those undergoing any screening (63% vs. 37%, p=0.036). Conclusions: While overall FMD screening in SCAD patients has increased, complete screening rates remain low. FMD was diagnosed more often in patients undergoing complete screening, highlighting the importance of head, neck, and abdominal imaging in all patients with SCAD.

Pregnancy-Related Complications in Patients With Fibromuscular Dysplasia: A Report From the European/International Fibromuscular Dysplasia Registry.

Current literature suggests a higher risk of pregnancy-related complications in patients with renal fibromuscular dysplasia (FMD). The aim of our study was to assess the nature and prevalence of pregnancy-related complications in patients subsequently diagnosed with FMD. A call for participation was sent to centers contributing to the European/International FMD Registry. Patients with at least 1 pregnancy were included. Data on pregnancy were collected through medical files and FMD characteristics through the European/International FMD Registry. Data from 534 pregnancies were obtained in 237 patients. Despite the fact that, in 96% of cases, FMD was not diagnosed before pregnancy, 40% of women (n=93) experienced pregnancy-related complications, mostly gestational hypertension (25%) and preterm birth (20%), while preeclampsia was reported in only 7.5%. Only 1 patient experienced arterial dissection and another patient an aneurysm rupture. When compared with patients without pregnancy-related complications, patients with complicated pregnancies were younger at FMD diagnosis (43 versus 51 years old; P<0.001) and had a lower prevalence of cerebrovascular FMD (30% versus 52%; P=0.003) but underwent more often renal revascularization (63% versus 40%, P<0.001). In conclusion, the prevalence of pregnancy-related complications such as gestational hypertension and preterm birth was high in patients with FMD, probably related to the severity of renal FMD. However, the prevalence of preeclampsia and arterial complications was low/moderate. These findings emphasize the need to screen hypertensive women for FMD to ensure revascularization before pregnancy if indicated and appropriate follow-up during pregnancy, without discouraging patients with FMD from considering pregnancy.