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Fibromuscular dysplasia

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https://www.readbyqxmd.com/read/28815187/a-rare-presentation-of-fibromuscular-dysplasia-postpartum-vascular-catastrophe-and-brief-literature-review
#1
Fatima Khan, Ali Raza Ghani, Larami Mackenzie, Ashwin Matthew, Usman Sarwar, Bruce Klugherz
Spontaneous coronary artery dissection is a very rare cause of acute coronary syndromes and can be life threatening given the rarity of the condition. It should be part of differentials in young females presenting with acute coronary syndromes without routine risk factors for coronary artery disease, especially before, during, and after pregnancy. It is closely associated with fibromuscular dysplasia and management can be very challenging at times. We present a case of spontaneous coronary artery dissection presenting with recurrent ST segment elevation myocardial infarction in association with fibromuscular dysplasia...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28804589/spontaneous-coronary-artery-dissection-an-under-recognized-cause-of-acute-coronary-syndromes
#2
Hanan Al Naabi, Hatim Al Lawati
Spontaneous coronary artery dissection (SCAD) is a rare condition that is often underdiagnosed given limitations of conventional cineangiography. In addition to the diagnostic challenge, the condition poses a major therapeutic dilemma given paucity of literature to guide management. We report the case of a 55-year-old woman, who presented with acute coronary syndrome. Coronary angiography at the time of the index hospitalization revealed type 2 SCAD. She was managed conservatively. Repeat coronary angiography three months later showed complete resolution of the previously noted dissection...
July 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28753427/a-genetic-variant-associated-with-five-vascular-diseases-is-a-distal-regulator-of-endothelin-1-gene-expression
#3
Rajat M Gupta, Joseph Hadaya, Aditi Trehan, Seyedeh M Zekavat, Carolina Roselli, Derek Klarin, Connor A Emdin, Catharina R E Hilvering, Valerio Bianchi, Christian Mueller, Amit V Khera, Russell J H Ryan, Jesse M Engreitz, Robbyn Issner, Noam Shoresh, Charles B Epstein, Wouter de Laat, Jonathan D Brown, Renate B Schnabel, Bradley E Bernstein, Sekar Kathiresan
Genome-wide association studies (GWASs) implicate the PHACTR1 locus (6p24) in risk for five vascular diseases, including coronary artery disease, migraine headache, cervical artery dissection, fibromuscular dysplasia, and hypertension. Through genetic fine mapping, we prioritized rs9349379, a common SNP in the third intron of the PHACTR1 gene, as the putative causal variant. Epigenomic data from human tissue revealed an enhancer signature at rs9349379 exclusively in aorta, suggesting a regulatory function for this SNP in the vasculature...
July 27, 2017: Cell
https://www.readbyqxmd.com/read/28747075/current-management-of-spontaneous-coronary-artery-dissection
#4
Teresa Bastante, Javier Cuesta, Marcos García-Guimaraes, Fernando Rivero, Ramon Maruri, David Adlan, Fernando Alfonso
Spontaneous coronary artery dissection (SCAD) remains an infrequent and elusive clinical entity of unknown etiology. However, our knowledge of SCAD has been significantly enriched in recent years. Large and prospective contemporary series have increased the interest in this disease with fewer patients misdiagnosed and a growing number of cases recognized in daily clinical practice. Classically, SCAD was thought to present mainly in young women without traditional atherosclerotic risk factors but, actually, most patients are middle-aged and are not free from coronary risk factors...
August 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28735951/presentation-clinical-features-and-results-of-intervention-in-upper-extremity-fibromuscular-dysplasia
#5
MULTICENTER STUDY
Nancy Nguyen, Aditya Sharma, Jonathan K West, Maya Serhal, Ellen Brinza, Heather L Gornik, Esther S H Kim
BACKGROUND: We present a case series of upper extremity fibromuscular dysplasia (UE FMD) consisting of 22 patients from two tertiary referral centers focusing on clinical presentation, diagnostic findings, and interventional outcomes. FMD is a noninflammatory, nonatherosclerotic arteriopathy that has a predisposition for middle-aged women. Involvement of the UE is thought to be rare. Patients with UE FMD can present with claudication or ischemia, or they can be incidentally diagnosed...
August 2017: Journal of Vascular Surgery
https://www.readbyqxmd.com/read/28728686/spontaneous-coronary-artery-dissection-associated-with-pregnancy
#6
Marysia S Tweet, Sharonne N Hayes, Elisabeth Codsi, Rajiv Gulati, Carl H Rose, Patricia J M Best
BACKGROUND: Spontaneous coronary artery dissection (SCAD) is the most common cause of pregnancy-associated myocardial infarction and remains poorly characterized. OBJECTIVES: This study sought to assess presentation, clinical factors, and outcomes of pregnancy-associated spontaneous coronary artery dissection (P-SCAD) compared with spontaneous coronary artery dissection not associated with pregnancy (NP-SCAD). METHODS: A Mayo Clinic registry was established in 2010 to include comprehensive retrospective and prospective SCAD data...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28722365/-segmental-arterial-mediolysis-a-differential-diagnosis-of-vasculitis
#7
Yassaman Alipour Tehrany, Sylvain Terraz, Jörg D Seebach
Segmental arterial mediolysis (SAM) is a rare non-atherosclerotic, non-inflammatory disease of unknown aetiology mostly affecting medium-sized arteries of the splanchnic circulation. Histologically, SAM is characterized by vacuolization and lysis of the arterial media leading to dissection, stenosis/occlusion or aneurysms. Symptoms vary from unspecific abdominal pain to hemorrhagic shock due to vessel rupture. The clinical and radiological presentation can mimic vasculitis, fibromuscular dysplasia or collagen vascular diseases...
April 5, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28716991/expanding-clinical-phenotype-of-fibromuscular-dysplasia
#8
EDITORIAL
Jeffrey W Olin
No abstract text is available yet for this article.
July 17, 2017: Hypertension
https://www.readbyqxmd.com/read/28716989/high-prevalence-of-multiple-arterial-bed-lesions-in-patients-with-fibromuscular-dysplasia-the-arcadia-registry-assessment-of-renal-and-cervical-artery-dysplasia
#9
Pierre-François Plouin, Jean-Philippe Baguet, Frédéric Thony, Olivier Ormezzano, Arshid Azarine, François Silhol, Catherine Oppenheim, Béatrice Bouhanick, Louis Boyer, Alexandre Persu, Frank Hammer, Philippe Gosse, Claire Mounier-Vehier, Claire Le Hello, Xavier Jeunemaitre, Michel Azizi, Laurence Amar, Gilles Chatellier, Elie Mousseaux, Emmanuel Touzé
Fibromuscular dysplasia (FMD) commonly affects the renal and cervical arteries but has been described to affect other vascular beds as well. The prevalence of and clinical characteristics associated with multisite FMD (string-of-beds or focal stenoses affecting at least 2 vascular beds) are not known. In the prospective ARCADIA registry (Assessment of Renal and Cervical Artery Dysplasia), symptomatic patients with renal artery (RA) FMD underwent tomographic- or magnetic resonance-angiography from the aortic arch to the intracranial arteries and those with cervical FMD from the diaphragm to the pelvis...
July 17, 2017: Hypertension
https://www.readbyqxmd.com/read/28715558/prevalence-of-intracranial-aneurysm-in-women-with-fibromuscular-dysplasia-a-report-from-the-us-registry-for-fibromuscular-dysplasia
#10
Henry D Lather, Heather L Gornik, Jeffrey W Olin, Xiaokui Gu, Steven T Heidt, Esther S H Kim, Daniella Kadian-Dodov, Aditya Sharma, Bruce Gray, Michael R Jaff, Yung-Wei Chi, Pamela Mace, Eva Kline-Rogers, James B Froehlich
Importance: The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. Objective: To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. Design, Setting, and Participants: This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers...
July 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28680766/cerebrovascular-fibromuscular-dysplasia-the-mgh-cohort-and-literature-review
#11
Andrea M Harriott, Eli Zimmerman, Aneesh B Singhal, Michael R Jaff, Mark E Lindsay, Guy A Rordorf
BACKGROUND: Fibromuscular dysplasia (FMD) is a rare noninflammatory, nonatherosclerotic arteriopathy of medium-sized arteries affecting up to 7% of the population. The disease can affect any artery but commonly affects renal, extracranial carotid, and vertebral arteries. The epidemiology and natural course of cerebrovascular FMD is unknown and requires further investigation. METHODS: We present demographic and outcomes data on a case series of 81 patients with cerebrovascular FMD from Massachusetts General Hospital presenting between 2011 and 2015 followed by a review of the peer-reviewed literature...
June 2017: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/28657981/lateralization-of-renal-blood-flow-and-renin-secretion-in-fibromuscular-dysplasia
#12
Masayuki Tanemoto
No abstract text is available yet for this article.
August 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28657457/the-spectrum-of-spontaneous-coronary-artery-dissection-illustrated-review-of-the-literature
#13
Marian Vandamme, Julie De Backer, Tine De Backer, Benny Drieghe, Daniel Devos, Sofie Gevaert
Spontaneous coronary artery dissection or SCAD is a rare and challenging disease that is increasingly diagnosed. It is characterized by a non-traumatic, non-iatrogenic separation of the coronary artery wall and occurs predominantly in young and middle-aged women without traditional cardiovascular risk factors. SCAD is often associated with predisposing conditions such as the peripartum period, systemic inflammatory disease and heritable connective tissue disease. More recently, independent investigators have demonstrated an important association with fibromuscular dysplasia...
June 28, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28652952/fibromuscular-dysplasia-with-spontaneous-coronary-artery-disease-presenting-as-acute-myocardial-infarction
#14
Ali Farooq, Waseem Amjad, Ata Ur Rahim Bajwa, Hassaan Yasin, Rizwan Ali, Muhammad Pervaiz
A 40-year-old female presented to a rural hospital with crushing substernal chest pain. An initial electrocardiogram showed ST elevation in lead II and aVF with elevated troponin I. She was immediately transferred to a tertiary care hospital. An emergent coronary angiogram did not show any significant coronary artery disease. On the second day, the patient experienced recurrence of severe chest pain with ST elevations in leads I, aVL, V5-V6, ST depressions in V1-V3, T-wave inversion over V2-V5. The troponin I level increased to > 40 ng/ml (normal 0...
May 23, 2017: Curēus
https://www.readbyqxmd.com/read/28622069/spontaneous-coronary-artery-dissection-in-a-woman-with-a-past-medical-history-of-subarachnoid-hemorrhage-a-case-report
#15
Jean Bardon, Fabien Picard, Franck Barbou, Olivier Varenne, Benoît Vivien
Spontaneous coronary artery dissection (SCAD) is an uncommon disease. We report the case of a 50 year-old woman with a past medical history of aneurysmal subarachnoid hemorrhage, presenting with acute chest pain and diffuse ST segment elevation on ECG. Coronary angiogram revealed a SCAD of the left anterior descending coronary artery. The association between cerebral aneurysms and SCAD should trigger providers concern for fibromuscular dysplasia. We hereby report on a rare and atypical case involving the relationship between fibromuscular dysplasia and SCAD...
June 16, 2017: Prehospital Emergency Care
https://www.readbyqxmd.com/read/28574637/efficacy-and-safety-of-gadobutrol-enhanced-mra-of-the-renal-arteries-results-from-grams-gadobutrol-enhanced-renal-artery-mra-study-a-prospective-intraindividual-multicenter-phase-3-blinded-study
#16
Kelly E Fabrega-Foster, Sheela Agarwal, Neda Rastegar, Daniel Haverstock, Jacob M Agris, Ihab R Kamel
PURPOSE: To compare the performance of magnetic resonance angiography (MRA) with 1M gadobutrol, a high relaxivity macrocyclic contrast agent, to 2D time-of-flight MRA (ToF-MRA) using computed tomographic angiography (CTA) as the standard of reference. Primary objectives were evaluation for superiority of structural delineation and noninferiority for detection and exclusion of clinically significant disease. MATERIALS AND METHODS: In all, 315 subjects underwent unenhanced and contrast-enhanced MRA with 1M gadobutrol (CE-MRA) and were scanned with 1...
June 2, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28560752/selective-embolization-therapy-for-intrarenal-artery-stenosis-causing-renovascular-hypertension-efficacy-and-follow-up-renal-imaging
#17
Eikan Mishima, Takehiro Suzuki, Kazumasa Seiji, Yasutoshi Akiyama, Hideki Ota, Junichiro Hashimoto, Kei Takase, Takaaki Abe, Sadayoshi Ito
We report the case of a young woman treated with selective renal embolization for renovascular hypertension caused by intrarenal artery stenosis and show follow-up imaging of the treated kidney. An 18-year-old woman had renin-dependent hypertension with intrarenal artery stenosis caused by fibromuscular dysplasia. A middle branch artery was nearly occluded, resulting in segmental renal ischemia with excessive renin secretion. Because our angioplasty attempt for revascularization failed as a result of technical difficulty, we performed selective embolization of the diseased vessel by anhydrous ethanol...
May 30, 2017: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/28497183/rapid-contralateral-progression-of-focal-cerebral-arteriopathy-distinguished-from-rnf213-related-moyamoya-disease-and-fibromuscular-dysplasia
#18
Yoshio Araki, Yasushi Takagi, Yohei Mineharu, Hatasu Kobayashi, Susumu Miyamoto, Toshihiko Wakabayashi
BACKGROUND: Focal cerebral arteriopathy includes unifocal or multifocal lesions that are unilateral or bilateral. Large- and/or medium-sized vessels are involved and can be visualized on angiography. CASE REPORT: We report a case of cerebral infarction in a 9-year-old Japanese female who presented with a transient ischemic attack. Steno-occlusion involving the distal part of the internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery was observed...
August 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28479775/surgical-revascularization-of-bilateral-renal-artery-stenosis-due-to-fibromuscular-dysplasia
#19
Chandan Phukan, Arun J P George, J Chandrasingh, Antony Devasia
Fibromuscular dysplasia (FMD) is a noninflammatory disease affecting small- and medium-sized arteries of the renal and the carotids. It affects the renal arteries in nearly 60%-75% cases. The primary clinical manifestation of renal FMD is hypertension. Medial fibroplasia represents the most common dysplastic lesion. We report two cases who presented with hypertension and renal insufficiency and on evaluation was found to have bilateral renal artery stenosis. Stenting of the renal vessels was not possible due to the narrowed caliber of the vessel and inability to cannulate the renal arteries...
April 2017: Urology Annals
https://www.readbyqxmd.com/read/28479449/symptomatic-carotid-bulb-atypical-fibromuscular-dysplasia
#20
Dorian Verscheure, Dominique Fabre, Didier Smadja, Elie Fadel
Fibromuscular dysplasia (FMD) is known as rare cause ischemic stroke and usually involves the internal carotid or vertebral arteries in middle age women. A poorly known type of FMD located at the carotid bulb is called atypical fibromuscular dysplasia. We present the case of 3 young black patients suffering from recurrent ischemic strokes in the same middle cerebral artery territory. Computed tomographic angiography and arteriography showed a moderate diaphragmatic stenosis of the carotid bulb. After surgical removal of the dysplasia, postoperative course was uneventful without any recurrence...
August 2017: Annals of Vascular Surgery
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