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Blood Press 2018 Dec 18:1-8. Epub 2018 Dec 18.

b Department of Hypertension , Institute of Cardiology , Warsaw , Poland.

Purpose: Smoking was identified as a potential factor contributing to fibromuscular dysplasia (FMD). To evaluate the prevalence of smoking and clinical characteristics in FMD subjects.

Material And Methods: We analysed 190 patients with confirmed FMD in at least one vascular bed. Read More

World J Nephrol 2018 Nov;7(7):143-147

Division of Nephrology, Department of Medicine, Tygerberg Hospital and Stellenbosch University, Cape Town 7505, South Africa.

Renal artery stenosis is a common cause of secondary hypertension and chronic kidney disease. We present here a case of fibromuscular dysplasia that was treated with surgical revascularization, resulting in recovery of kidney function with eventual cessation of chronic dialysis. The case involves a 25-year-old female with coincidentally discovered hypertension, who underwent further investigations revealing a diagnosis of renal artery stenosis due to fibromuscular dysplasia. Read More

Ann Vasc Surg 2018 Nov 27. Epub 2018 Nov 27.

UNC Division of Vascular Surgery, Chapel Hill (NC).

Fibromuscular dysplasia (FMD) is a non-atherosclerotic disease that generally affects medium-sized arteries. The distribution typically involves the renal, extracranial carotid/vertebral, and iliac arteries. FMD in other vascular beds is rare. Read More

Lancet 2018 Nov;392(10161):2298

Department of Medicine, Division of Nephrology, Hospital Costa del Sol, Marbella, Malaga, Spain.

Radiol Case Rep 2019 Feb 20;14(2):242-245. Epub 2018 Nov 20.

Department of Diagnostic Imaging and Interventional Radiology, University of Tor Vergata, Viale Oxford, 81, Rome 00133, Italy.

Renal artery stenosis commonly manifests with hypertension refractory to medical treatment. We report a case of renal artery stenosis occurring in a 19-year-old female patient who presented with extremely high blood pressure and bilateral renal stenosis at the duplex ultrasound. Renal angiography confirmed the bilateral and irregular stenosis due to fibromuscular dysplasia, associated to extent collaterals suppling the poststenotic right renal artery. Read More

Am J Cardiol 2018 Oct 30. Epub 2018 Oct 30.

Brigham and Women's Hospital Heart & Vascular Center, Harvard Medical School, Boston, Massachusetts. Electronic address:

The pathophysiology of spontaneous coronary artery dissection (SCAD) is heterogeneous, associated with systemic arteriopathies and inflammatory diseases, and often compounded by environmental precipitants, genetics, or stressors. However, the frequency of these associated conditions with SCAD on a population level remains unknown. Therefore, the objective of this analysis was to evaluate heterogeneous phenotypes of SCAD in the United States using data from the Nationwide Inpatient Sample collected from January 1, 2004, to September 31, 2015. Read More

Catheter Cardiovasc Interv 2018 Nov 23. Epub 2018 Nov 23.

Division of Cardiology, Vancouver General Hospital, University of British Columbia, Canada.

Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome especially in women. The most common underlying predisposing cause of SCAD is fibromuscular dysplasia (FMD), a non-inflammatory arteriopathy that results in weakening of the affected arteries, and can cause dissection or aneurysm. Coronary FMD (CFMD) was described as rare, and was shown to cause SCAD in histopathological case reports. Read More

Int J Cardiol 2018 Nov 9. Epub 2018 Nov 9.

Department of Cardiovascular Sciences, University of Leicester and NIHR Leicester Biomedical Research Centre, Glenfield Hospital, Leicester, UK.

Spontaneous coronary artery dissections (SCADs) are increasingly recognized as an important cause of acute coronary syndromes in predominantly women below 60 years of age. SCAD patients comprise a heterogeneous group, in which it is estimated that a quarter to one third have underlying fibromuscular dysplasia (FMD). Although the mutual relationship of SCAD and FMD is complex and only partly understood, there seems to be some overlap in genetic background and interaction with endogenous sex-steroids. Read More

Medicine (Baltimore) 2018 Nov;97(46):e12815

Department of Nephrology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Rationale: Fibromuscular dysplasia (FMD) is a rare vascular disorder that causes abnormal cell growth in arterial walls. The classic "string of beads" sign has been reported in many cases, whereas the appearance of tubular stenosis and distal tapering of renal arteries with multiple renal infarctions, as well as left kidney atrophy occurring in one patient, has not been precisely described.

Patient Concerns: A 19-year-old woman presented to us with a history of elevated blood pressure without any symptoms for the past 1 month. Read More

Curr Treat Options Cardiovasc Med 2018 Nov 13;20(12):103. Epub 2018 Nov 13.

Adult Congenital and Cardiovascular Genetics Center, Division of Cardiovascular Medicine, University of Minnesota Medical Center, Minneapolis, MN, USA.

Purpose Of Review: Numerous studies have begun to unravel the genetic basis of not only aortic disease but also other forms of commonly encountered vascular diseases. The goal of this review is to provide clinicians a reference to help identify and diagnose different types of vascular disease with a genetic underpinning.

Recent Findings: Ongoing studies have identified numerous genes involved in the TGF-β signaling pathway that are also associated with thoracic aortic aneurysm and dissection, and it is possible to test for pathogenic variants in these genes in the clinical setting using commercially available genetic testing panels. Read More

J Obstet Gynaecol 2018 Nov 10:1-3. Epub 2018 Nov 10.

a The Women and Children's Hospital , Hull Royal Infirmary , Hull , UK.

Can J Cardiol 2018 Dec 17;34(12):1689.e3-1689.e4. Epub 2018 Sep 17.

Department of Cardiology, Tokyo Saiseikai Central Hospital, Tokyo, Japan.

Interv Neurol 2018 Oct 11;7(6):413-418. Epub 2018 Jul 11.

Emory University/Grady Memorial Hospital, Atlanta, Georgia, USA.

Background: A carotid web (CaW) is a shelf-like lesion in the posterior aspect of the internal carotid bulb and represents an intimal variant of fibromuscular dysplasia. CaW has been associated with recurrent strokes and conventionally treated with surgical excision. We report a multicenter experience of stenting in patients with symptomatic CaWs. Read More

Ann Vasc Dis 2018 Sep;11(3):365-368

Department of Surgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan.

Fibromuscular dysplasia (FMD) mainly develops in medium-sized arteries, including renal, extracranial, and extremity arteries, but it rarely causes abdominal aortic aneurysm (AAA). A 69-year-old woman with AAA diagnosed on ultrasonography by a home doctor visited our hospital. Contrast-enhanced computed tomography revealed a saccular aneurysm of terminal abdominal aorta. Read More

JAMA Neurol 2018 Nov 5. Epub 2018 Nov 5.

Department of Neurology, Emory University Hospital and Marcus Stroke and Neuroscience Center, Grady Memorial Hospital, Atlanta, Georgia.

Importance: A succession of research has explored the linkage between carotid webs (CaWs) and ischemic strokes. Imaging and pathologic analysis have defined CaW as an intimal variant of fibromuscular dysplasia, which appears as a shelflike lesion on the posterior aspect of the carotid bulb, more specifically at the origin of internal carotid artery. Reported findings of carotid webs in young patients with recurrent ischemic strokes without an otherwise determined cause have raised questions about the mechanism, natural history, and need for intervention. Read More

Tex Heart Inst J 2018 08 1;45(4):233-235. Epub 2018 Aug 1.

BJR Case Rep 2018 9;4(1):20170066. Epub 2017 Nov 9.

Department of Stroke Medicine, Mater Misericordiae University Hospital, Dublin, Ireland.

Carotid webs are an uncommon cause of recurrent ischaemic strokes. They are considered a form of fibromuscular dysplasia, possibly developmental in origin, with non-inflammatory and non-atherosclerotic features and a characteristic appearance on CT angiography. They have been described as a thin intraluminal filling defect along the posterior wall of the carotid bulb in oblique sagittal reformats and a septum on axial CT angiography. Read More

Eur J Vasc Endovasc Surg 2018 Oct 18. Epub 2018 Oct 18.

Department of Surgical Sciences, Vascular Surgery, Uppsala University, Uppsala, Sweden.

Objective/background: To study functional outcome, mortality, and dialysis free survival in patients undergoing open primary surgical repair of renal artery stenosis (RAS) or aneurysm (RAA).

Methods: This was a retrospective single centre study of patients undergoing open surgical renal artery reconstruction from 1993 to 2007. Blood pressure, renal function, dialysis dependence, vessel patency, and mortality were registered. Read More

World Neurosurg 2019 Jan 12;121:e925-e930. Epub 2018 Oct 12.

Department of Neurosurgery, Louisiana State University Health Sciences Center Shreveport, Shreveport, Louisiana, USA.

Background: The increasing frequency of elderly patients with severe tortuous anatomy, especially when combined with fibromuscular dysplasia, can make intracranial canalization severely difficult or impossible. Computed tomography angiography was used to determine the feasibility of accessing the internal carotid artery (ICA) via a percutaneous translacerum approach.

Methods: Twenty consecutive stroke activations with CT angiography were reconstructed in three-dimensional models to take measurements to assess if currently available technologies could safely provide access. Read More

Am J Med 2018 Oct 2. Epub 2018 Oct 2.

Department of Medicine, University of California, San Francisco; Medical Service, VA Medical Center, San Francisco, Calif.

JAMA Neurol 2018 Oct 1. Epub 2018 Oct 1.

Paris-Descartes University, Institut National de la Santé et de la Recherche Médicale UMR970, Assistance-Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Hypertension Unit, Paris, France.

Importance: Data on neurologic manifestations of fibromuscular dysplasia (FMD) are rare, and current knowledge remains limited.

Objectives: To present a comprehensive review of the epidemiologic characteristics, management, and prognosis of the neurologic manifestations associated with cerebrovascular FMD (ie, involving cervical or intracranial arteries) and to guide future research priorities.

Evidence Review: References were identified through searches of PubMed from inception to December 2017 using both the medical subject headings and text words. Read More

J Cardiol Cases 2017 Dec 19;16(6):216-218. Epub 2017 Sep 19.

Department of Cardiology, University Hospital of Salamanca, Salamanca, Spain.

Spontaneous coronary artery dissection (SCAD) usually appears as an acute coronary syndrome with good prognosis. We present the case of a 39-year-old woman with progressive SCAD secondary to fibromuscular dysplasia with catastrophic course. The patient required several mechanical circulatory support systems including a left ventricular assist device (CentriMag, Thoratec, Pleasanton, CA, USA) as bridge to recovery. Read More

J Vasc Surg 2018 10;68(4):1226-1227

Division of Vascular and Endovascular Surgery, UMass Memorial Medical Center, University of Massachusetts Medical School, Worcester, Mass.

Vasc Endovascular Surg 2018 Sep 17:1538574418800557. Epub 2018 Sep 17.

1 Vascular surgery, OhioHealth Doctors Hospital, Columbus, OH, USA.

True renal artery aneurysms are rare. Ruptured aneurysms are even rarer but can have devastating consequences. Renal artery aneurysms most commonly occur in patients with hypertension, atherosclerosis, or fibromuscular dysplasia. Read More

J Med Vasc 2018 Sep 23;43(5):329-331. Epub 2018 Jul 23.

Department of Internal Medicine and Infectious diseases, Centre Hospitalier Marc-Jacquet, 77000 Melun, France.

A 50-year-old man had a sudden severe abdominal pain. An abdominal enhanced computed tomographic scan revealed irregular stenosis of the celiac artery, dissection of the hepatic artery and of the left common iliac artery, and mid stenosis of the right renal artery with extended renal parenchymal hypodensity. After careful evaluation, the patient was diagnosed with fibromuscular dysplasia. Read More

J Vasc Surg Cases Innov Tech 2018 Sep 29;4(3):232-236. Epub 2018 Aug 29.

Department of Radiology, A.V. Vishnevsky Medical Research Center of Surgery of the Ministry of Healthcare of the Russian Federation, Moscow, Russian Federation.

Renal arteriovenous fistulas are pathologic communications between the renal arteries and veins without interconnecting capillaries. Reports on the combination of fibromuscular dysplasia, aneurysms, and renal arteriovenous fistula are extremely rare in the literature. In the case of renal arteriovenous aneurysm rupture, urgent nephrectomy was the only life-saving procedure reported. Read More

J Thorac Dis 2018 Jul;10(7):4602-4608

Department of Cardiology, Hospital Universitario de La Princesa, Madrid, Spain.

Spontaneous coronary artery dissection (SCAD) represents an increasingly recognized cause of acute coronary syndrome (ACS), especially in young females. Novel diagnostic insights obtained from intracoronary imaging and the frequent association with fibromuscular dysplasia (FMD), have recently enriched our understanding of this unique clinical entity. Recently, two scientific statements from both sides of the Atlantic, summarizing the available information on SCAD, have been simultaneously published. Read More

World J Gastroenterol 2018 Aug;24(32):3637-3649

Division of Gastroenterology and Hepatology, Graduate School of Medical and Dental Sciences, Niigata University, Niigata 9518510, Japan.

The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the development and improvement of imaging modalities, diagnostic frequency of these vascular diseases in abdominal lesions is increasing even with the small changes in the vasculatures. Among various vascular diseases, fibromuscular dysplasia (FMD) and segmental arterial mediolysis (SAM) are noninflammatory, nonatherosclerotic arterial diseases which need to be diagnosed urgently because these diseases could affect various organs and be lethal if the appropriate management is not provided. Read More

Eur J Clin Invest 2018 Nov 17;48(11):e13023. Epub 2018 Sep 17.

Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. Read More

Vasc Endovascular Surg 2019 Jan 16;53(1):66-70. Epub 2018 Aug 16.

2 Division of Vascular Surgery, Stanford University, Stanford, CA, USA.

We describe successful endovascular treatment of a patient with fibromuscular dysplasia of the celiac axis leading to development of a common hepatic artery pseudoaneurysm with contained rupture. An 81-year-old woman was transferred to our quaternary care center with concern for a hepatic artery rupture. Further imaging demonstrated a common hepatic artery pseudoaneurysm with surrounding hematoma as well as multifocal areas of narrowing and dilatation in the celiac trunk consistent with fibromuscular dysplasia. Read More

Mol Genet Metab Rep 2018 Sep 26;16:64-65. Epub 2018 Jul 26.

Centre de Référence Neuromusculaire Nord/Est/Ile de France, AP-HP, Institut de Myologie, GH Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75013 Paris, France.

Vascular involvement in Late Onset Pompe Disease, glycogen storage disease type II characterized by limb-girdle muscle and diaphragmatic weakness, is well documented. Abnormalities of posterior cerebral circulation have mostly been reported, whereas there are also cases of associated extracerebral arteriopathy. We report the case of a 42-year-old man diagnosed with LOPD a year after renal infarct due to renal artery fibromuscular dysplasia. Read More

Cureus 2018 Mar 14;10(3):e2326. Epub 2018 Mar 14.

Department of Hematology and Oncology, Fairview Hospital, Cleveland Clinic, Cleveland, USA.

Fibromuscular dysplasia (FMD) is a condition caused by an abnormal development or growth of cells in the arterial walls in the body. We present a case of a 49-year-old male who came in with a sudden onset of severe left-sided abdominal pain. Computed tomography (CT) scan of the abdomen was suggestive of a left renal infarct. Read More

Cerebrovasc Dis 2018 31;46(1-2):33-39. Epub 2018 Jul 31.

Background And Purpose: Fibromuscular dysplasia (FMD) is a non-atherosclerotic arteriopathy most often affecting the carotid and renal arteries. In the United States Registry for FMD, 41.7% of patients experienced an aneurysm and/or dissection by the time of entry into the Registry. Read More

Medicine (Baltimore) 2018 Jul;97(30):e11538

Department of Neurology, the Affiliated Hospital of Inner Mongolia Medical University, Huhhot, Inner Mongolia.

Introduction: Fibromuscular dysplasia (FMD) is a rare and controversial disease that is seldom associated with genes. Here, we report the discovery of 2 missense mutations in COL4A5 and COL4A6 that may be risk factors for causing cerebrovascular FMD. We performed high-throughput sequencing on a patient with FMD and her probable healthy daughter, then annotated the frequency of a variant in a control or general population and assessed its deleterious effects according to published guidelines. Read More

J Clin Hypertens (Greenwich) 2018 Sep 19;20(9):1302-1309. Epub 2018 Jul 19.

Department of Cardiology, National Center for Cardiovascular Diseases, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

The time trends regarding the etiology of renal artery stenosis (RAS) are changing, but few investigations have focused on these issues. This study aimed to analyze the time trends regarding the etiology of RAS in a large patient sample from the China Center for Cardiovascular Disease. Consecutive inpatients with RAS from January 1999 to December 2016 were enrolled in this study. Read More

Postepy Kardiol Interwencyjnej 2018 19;14(2):214-215. Epub 2018 Jun 19.

Department of Nephrology, Jagiellonian University Medical College, Krakow, Poland.

EJVES Short Rep 2018 8;39:16-19. Epub 2018 May 8.

York and North Yorkshire Vascular Unit, York Teaching Hospital NHS Foundation Trust, York, UK.

Introduction: Fibromuscular dysplasia (FMD) and superior mesenteric artery (SMA) aneurysms are rare vascular conditions. An unusual combination of both diseases is reported.

Case Report: A 54 year old woman presented with symptomatic SMA aneurysm. Read More

J Med Vasc 2018 Jul;43(4):262-266

Department of cardiovascular and thoracic surgery, Habib Bourguiba hospital, 3029 Sfax, Tunisia.

Extracranial carotid artery aneurysms are rare. The most common location is the common carotid artery near the bifurcation. The mid to distal internal carotid artery is the second most common location. Read More

Hypertens Res 2018 Sep 3;41(9):639-648. Epub 2018 Jul 3.

Department of Internal Medicine and Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Medical Centre (MUMC+), Maastricht, The Netherlands.

Fibromuscular dysplasia is a heterogeneous group of systemic, noninflammatory, and nonatherosclerotic diseases of the vascular wall. It is the second-most common abnormality of the renal artery. Although hypertension is the most common presenting symptom, other symptoms, such as pulsatile tinnitus, stroke, chest pain, or abdominal discomfort, may result from other affected vascular beds. Read More

Heart 2018 Oct 28;104(19):1607. Epub 2018 Jun 28.

Monash Cardiovascular Research Centre, Monash University, Clayton, Victoria, Australia.

Clinical Introduction: A 42-year-old woman presented with anterior ST elevation myocardial infarction. Urgent coronary angiography revealed tapering then occlusion of the distal left anterior descending (LAD) coronary artery with no flow in the distal LAD (figure 1A). Balloon angioplasty with a 2. Read More

J Vasc Surg Cases Innov Tech 2018 Jun 12;4(2):76-79. Epub 2018 Apr 12.

Department of Vascular Surgery, Medical College of Wisconsin, Milwaukee, Wisc.

We report the case of a 44-year-old woman who developed an acute type B aortic dissection caused by an entry tear from an aneurysmal left common iliac artery that extended retrograde to the proximal descending thoracic aorta. She experienced refractory chest pain despite optimal medical management, thereby indicating repair. Endovascular aortic repair was subsequently performed. Read More

JAMA Cardiol 2018 Aug;3(8):756-760

Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, Ohio.

Importance: Fibromuscular dysplasia (FMD) is a nonatherosclerotic arterial disease that predominately affects women and is most commonly diagnosed in middle age. The natural history of FMD among patients diagnosed at an older age is not well understood.

Objective: To examine the differences in clinical presentation, arterial bed involvement, vascular events, and need for vascular procedures between younger and older patients with FMD. Read More

Cardiol Res 2018 Jun 6;9(3):195-196. Epub 2018 Jun 6.

Vascular Medicine and Vascular Diagnostic Laboratory, The Zena and Michael A. Wiener Cardiovascular Institute and Marie-Josee and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine at Mount Sinai, One Gustave L. Levy Pl, Box 1033, New York, NY 10029, USA.

Cardiol Res 2018 Jun 6;9(3):191-194. Epub 2018 Jun 6.

Department of Cardiology, St Mary Medical Center, Long Beach, CA 90813, USA.

Spontaneous coronary artery dissection (SCAD) is a variant of acute coronary syndrome (ACS) that is poorly understood. SCAD has been linked to fibromuscular dysplasia (FMD), connective tissue disease (CTD), pregnancy and hormonal imbalance, systemic inflammatory conditions (e.g. Read More

J Hypertens 2018 Aug;36(8):1729-1735

Department of Internal Medicine.

Objective: Fibromuscular dysplasia (FMD) can be classified in a multifocal and a unifocal subtype. As unifocal FMD generally leads to more severe hypertension at younger age, we hypothesized that renal hemodynamics are more disturbed in unifocal renal artery FMD as compared with multifocal FMD, leading to increased renin secretion.

Methods: We measured renal blood flow (Xenon washout method), renin secretion, and glomerular filtration rate per kidney in 101 patients with FMD (26 unifocal and 75 multifocal), all off medication and prior to balloon angioplasty. Read More

Int J Mol Sci 2018 May 21;19(5). Epub 2018 May 21.

Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 1200 Brussels, Belgium.

Fibromuscular Dysplasia (FMD) is “an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries” (Persu, et al; 2014). FMD can lead to hypertension, arterial dissections, subarachnoid haemorrhage, stroke or mesenteric ischemia. The pathophysiology of the disease remains elusive. Read More

Pediatr Nephrol 2018 Sep 4;33(9):1593-1599. Epub 2018 Jun 4.

Division of Nephrology, Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Background: Fibromuscular dysplasia (FMD) is a non-inflammatory vascular disease that in children unlike in adults shows no sex predilection. FMD is often underdiagnosed, and its pathophysiology is unclear. Delayed diagnosis may lead to refractory hypertension and decreases the chance of successful treatment. Read More

J Am Soc Hypertens 2018 Jul 9;12(7):506-508. Epub 2018 May 9.

Department of Interventional Radiology, Great Ormond Street Hospital for Children, London, UK.

Cardiovasc Pathol 2018 Jul - Aug;35:57-63. Epub 2018 Apr 30.

State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address: