3,192 results match your criteria: "Fibromuscular Dysplasia"

Contemporary review on spontaneous coronary artery dissection: insights into the angiographic finding and differential diagnosis.

Front Cardiovasc Med

November 2023

Faculty of Medical Sciences, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, United Kingdom.

Spontaneous coronary artery dissection (SCAD), although in the majority of cases presents as an acute coronary syndrome (ACS), has different pathophysiology from atherosclerosis that influences specific angiography findings and enables most patients to be solved by optimal medical therapy rather than percutaneous coronary intervention (PCI). Therefore, accurate diagnosis is essential for adequate treatment of each patient as management of SCAD differs from that of ACS of atherosclerotic aetiology. So far, invasive coronary angiography remains the most important diagnostic tool in suspected SCAD.

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

Vasc Med

February 2024

Division of Cardiology, Cliniques Universitaires Saint-Luc and Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium.

Introduction: Although renal stenting is the standard revascularization method for atherosclerotic renal artery stenosis (RAS) (FMD-RAS), stenting in fibromuscular dysplasia (FMD) RAS is usually limited to periprocedural complications of angioplasty and primary arterial dissection. The main aim of the study was to retrospectively analyze the immediate and long-term results of renal stenting versus angioplasty in patients with FMD.

Methods: Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries).

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We present a rare case of fibromuscular dysplasia (FMD) manifesting in the mid segment of right renal artery, which led to the development of refractory hypertension. The patient received balloon angioplasty to a severe lesion on the middle of right renal artery and subsequently had normalisation of blood pressures. Fractional flow reserve (FFR) detection of the renal artery before and after balloon dilatation was 0.

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Aim: The first expert consensus documents on management of patients with spontaneous coronary artery dissection (SCAD) were published in 2018. Worldwide quality of care, as measured by adherence to these recommendations, has not been systematically reviewed. We aim to review the proportion of patients with SCAD receiving consensus recommendations globally, regionally and, determine differences in practice before and after 2018.

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Cerebral Infarction and Recovery in a 12-Year-Old Child With Intracranial Fibromuscular Dysplasia.

Brain Neurorehabil

November 2023

Department of Rehabilitation Medicine, College of Medicine, Chungnam National University, Daejeon, Korea.

Fibromuscular dysplasia (FMD) is a congenital vascular anomaly resulting in arterial stenosis and weakening of typically medium-sized arteries. It is a noninflammatory, nonatherosclerotic arterial disease that affects most commonly the renal and internal carotid arteries, but intracranial FMD in the pediatric population is very rare. We report a young age-onset ischemic stroke patient with FMD affecting the middle cerebral artery (MCA).

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Background: Carotid web (CaW) is a subtype of fibromuscular dysplasia that predominantly involves the intimal layer of the arterial wall and is commonly overlooked as a separate causative entity for recurrent strokes. CaW is defined as a shelf-like lesion at the carotid bulb, although different morphological features have been reported. Optical coherence tomography (OCT) has been described in the literature as a useful microscopic and cross-sectional tomographic imaging tool.

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Article Synopsis
  • Rare vascular conditions often create challenges for healthcare providers, particularly in female patients, due to their low frequency and limited research.
  • A literature review focused on three such conditions—median arcuate ligament syndrome, fibromuscular dysplasia, and vascular Ehlers-Danlos syndrome—revealed significant sex-based differences in prevalence and outcomes.
  • The review also highlights important considerations for pregnant women dealing with these rare vascular conditions, emphasizing the need for more focused research in this area.
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Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic separation of the coronary artery wall with subsequent intramural hematoma (IMH) formation in the false lumen. It can be associated with or without an intimal tear. It is clinically divided into three types based on its angiographic appearance.

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Article Synopsis
  • Fibromuscular dysplasia (FMD) can lead to renovascular hypertension, particularly in young women who may not be adequately diagnosed.
  • A 20-year-old woman presented with high blood pressure and low potassium levels, ultimately diagnosed with branch artery FMD after imaging and tests showed significant renin secretion from the right kidney.
  • Following successful treatment with percutaneous transluminal angioplasty, she was cured of her hypertension and didn't need any medications, highlighting the importance of proper diagnosis and treatment in such cases.
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Segmental arterial mediolysis (SAM) is a rare vascular disease, characterized by acute but transient vulnerability of the wall of medium-sized arteries. The most characteristic feature of SAM is its biphasic course: an injurious phase marked by acute weakness of the arterial wall leading to acute dissection and/or hemorrhage, followed by a reparative phase in which granulation tissue and fibrosis restore the injured arterial wall. Residual stenosis, aneurysms, and/or arterial wall irregularities may remain visible on future imaging studies.

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Lesions of the Cardiac Conduction System and Sudden Death.

Am J Forensic Med Pathol

March 2024

From the Department of Pathology and Laboratory Medicine, Corewell Health, Grand Rapids, MI.

When a young previously healthy person dies suddenly, occasionally, the scene is noncontributory and the autopsy and drug screen are negative. In such cases, additional studies, including genetic assessment and cardiac conduction system examination, should be performed. We performed a literature search and reviewed our own material to identify possible or definite conduction system anomalies that may cause death.

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Advancements in the Genetics of Spontaneous Coronary Artery Dissection.

Curr Cardiol Rep

December 2023

Department of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, USA.

Purpose Of Review: Spontaneous coronary artery dissection (SCAD) is a significant cause of acute myocardial infarction that is increasingly recognized in young and middle-aged women. The etiology of SCAD is likely multifactorial and may include the interaction of environmental and individual factors. Here, we summarize the current understanding of the genetic factors contributing to the development of SCAD.

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Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a nonatheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months.

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Background: Carotid web (CaW) and carotid free-floating thrombus (CFFT) are rare yet critical causes of ischemic stroke in young adults.

Case Presentation: A 54-year-old woman presented with a fluctuating right sensory-motor faciobrachial syndrome. A brain MRI scan revealed multiple small recent asynchronous cortico-subcortical ischemic foci in the vascular territory of the left internal carotid artery.

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Spontaneous coronary artery dissection (SCAD) accounts for 1.7%-4% of all acute coronary syndrome presentations, particularly among young women with an emerging awareness of its importance. The demarcation of acute SCAD from coronary atherothrombosis and the proper therapeutic approach still represents a major clinical challenge.

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Fibromuscular dysplasia (FMD) is a noninflammatory arterial diseases that affects predominantly women. Multiple studies have demonstrated an increased prevalence of FMD in patients who experience carotid or vertebral artery dissection (VAD). This case report presents a 57-year-old female who presented with a headache and was diagnosed with partially thrombosed giant aneurysm of vertebral artery.

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[French National registry of spontaneous coronary artery dissections : ''DISCO registry''].

Ann Cardiol Angeiol (Paris)

December 2023

Service de cardiologie, Centre Hospitalier Universitaire Gabriel-Montpied, CNRS, Université Clermont Auvergne, Clermont-Ferrand, France.

Aim: Spontaneous 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.

Method: From 2016 to 2018, 424 patients with a diagnosis of SCAD were included prospectively and retrospectively in 51 French cardiology centres.

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In recent years, endovascular treatments have become more common in patients with renal artery stenosis and aneurysm. Although the treatment algorithms are not universally accepted, endovascular therapy can be readily utilized for the appropriate indications in the context of surgical treatment for renovascular diseases. The most important factor to consider is that the correct indication is applied for such treatment.

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Drug-coated balloon for treatment of non-atherosclerotic renal artery stenosis-a multi-center study.

BMC Cardiovasc Disord

October 2023

Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Shuaifuyuan 1st, Dongcheng District, Beijing, 100730, China.

Introduction: Renal artery stenosis (RAS) is a significant reason for secondary hypertension. Impaired renal function and subsequent cardiopulmonary dysfunction could also occur. Patients of non-atherosclerotic RAS has a relatively young age and long life expectancy.

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This review highlights sex- and gender-specific considerations in cardiovascular diseases with a particular focus on pathophysiology, epidemiology, clinical presentation, risk factors, diagnosis, and management of spontaneous coronary artery dissection. It aims to summarize the most up to date evidence and gaps in knowledge that exist in the spontaneous coronary artery dissection space while highlighting salient points with regards to sex- and gender-based differences in clinical implications, current management, and recommended counseling practices.

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Though an infrequent cause of acute coronary syndrome, spontaneous coronary artery dissection is an increasingly recognized cardiovascular condition predominantly seen in middle-aged females. Its pathophysiology is defined by separation of coronary arterial wall layers which cause acute coronary syndrome-like presentations with relatively high recurrence rates. Overall, there is a lack of reported literature and understanding of the short- and long-term management for spontaneous coronary artery dissection.

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Unique presentation of renovascular hypertension due to fibromuscular dysplasia.

J Vasc Surg Cases Innov Tech

September 2023

Division of Vascular and Endovascular Surgery, University of California, San Diego, La Jolla, CA.

A 21-year-old female with a history of right nephrectomy due to trauma presented with several years of multidrug-resistant hypertension. Her workup included negative findings from autoimmune and vasculitides panels and urine catecholamine testing. Computed tomography showed an acute hairpin turn of her left renal artery.

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Arterial dissection is the result of blood entering along the intima-media plane through a breach produced either spontaneously or traumatically. Cervical arterial dissections are an important cause of acute ischemic stroke in children, young adults, and patients with cranio-cervical traumatic injuries. Arterial dissections occur either spontaneously, in genetic diseases, the most important association being with fibromuscular dysplasia.

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Grange syndrome (GRNG-MIM#135580) is a rare recessive disorder associating variable features including diffuse vascular stenosis, brachysyndactyly, osteopenia with increased bone fragility, cardiac malformations, and variable developmental delay. Since its first description in 1998, only 15 individuals from 10 families have been reported, carrying homozygous or compound heterozygous frameshift or nonsense variants in YY1AP1. In a patient with cutaneous and bone syndactyly and a hemorrhagic stroke at the age of 16 months, consistent with a clinical diagnosis of GRNG, we performed exome sequencing after negative array-CGH and congenital limb malformation panel results.

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