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Vasc Endovascular Surg 2019 Apr 13;53(3):250-254. Epub 2018 Dec 13.

1 Department of Surgery, Fairfield Medical Center, Lancaster, OH, USA.

We describe a case of concomitant fibromuscular dysplasia (FMD) and atherosclerotic internal carotid artery (ICA) disease in a symptomatic patient. Sixty-eight-year-old female presented for evaluation of a transient ischemic attack. Imaging revealed severe proximal >80% ICA stenosis with severe FMD to mid and distal ICA. Read More

Am J Forensic Med Pathol 2019 Mar 5. Epub 2019 Mar 5.

Medico-Legal and Pathological-Anatomical Department of Health Care Surveillance Authority, Košice, Slovak Republic.

In this article, we report the autopsy findings of a 23-year-old woman, who was found unconscious at home by her relatives. During the transportation to the hospital, the woman was handed over to the ambulance personnel, who were the first to provide cardiopulmonary resuscitation. In the hospital, after an hour-lasting asystole, the heart activity was restored. Read More

JACC Cardiovasc Interv 2019 Feb 22. Epub 2019 Feb 22.

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

Objectives: Given the uncertainty regarding the degree and prevalence of spontaneous healing following spontaneous coronary artery dissection (SCAD), the aim of this study was to assess the angiographic characteristics of the dissected segments in a large cohort of patients with SCAD who underwent subsequent repeat coronary angiography.

Background: SCAD is an uncommon yet important cause of myocardial infarction in women. Very little is known about the characteristics of healing of dissected arteries. Read More

Tidsskr Nor Laegeforen 2019 Feb 19;139(4). Epub 2019 Feb 19.

Fibromuscular dysplasia affects the muscles of small and medium-sized arteries. The aetiology of the condition is unknown; it is most frequently seen in middle-aged women, but can affect both sexes at any age. Hypertension is the most common clinical manifestation when the renal arteries are affected. Read More

Vasc Med 2019 Feb 21:1358863X18818317. Epub 2019 Feb 21.

Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH, USA.

Urol Ann 2019 Jan-Mar;11(1):46-52

Department of Urology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India.

Introduction: With significant advances in the area of interventional radiology, angioplasty and stenting have become preferred first-line treatment in patients with significant renal artery stenosis. However, not all patients have favorable anatomy to undergo minimally invasive treatments, and reconstruction of the renal artery is an option. In select cases, either improved renal function or maintenance of existing function and sometimes resolution of hypertension can follow surgical treatment. Read More

Insights Imaging 2019 Feb 15;10(1):21. Epub 2019 Feb 15.

Department of Imaging Sciences, University of Rochester Medical Center, 601 Elmwood Avenue, P.O. Box 648, Rochester, NY, 14642, USA.

The complex anatomy of the carotid space within a small confined area is unique to the head and neck and allows for a vast array of pathology. This paper will review the anatomy of the carotid space from the skull base to the thorax, defining its borders at multiple levels, as well as its contents. The paper will also describe the myriad of mass lesions and vascular pathologies that may occur within the carotid space. Read More

Ann Vasc Surg 2019 Feb 13. Epub 2019 Feb 13.

Department of Surgery "Pietro Valdoni", "Sapienza" University of Rome, Rome, Italy.

Visceral aneurysms are rare entities, with a reported incidence of 0.01-2%. Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory artery disease, which may predispose to aneurysmal degeneration. Read More

Vasc Med 2019 Feb 10:1358863X18811596. Epub 2019 Feb 10.

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

The association between fibromuscular dysplasia (FMD) and spontaneous cervical artery dissection (SCeAD) has been recognized, but the available evidence on this relationship is scant. Therefore, the main goal of our study was to systematically evaluate FMD frequency, clinical characteristics and vascular bed involvement in patients with SCeAD. Among 230 patients referred to the ARCADIA-POL study, 43 patients (mean age 44. Read More

Vasc Med 2019 Feb 10:1358863X19826346. Epub 2019 Feb 10.

Zena and Michael A. Wiener Cardiovascular Institute, and Marie-Josée and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Am J Med Genet A 2019 Apr 1;179(4):552-560. Epub 2019 Feb 1.

National Institute on Aging Intramural Research Program, National Institutes of Health, Baltimore, Maryland.

We describe a data repository on heritable disorders of connective tissue (HDCT) assembled by the National Institutes of Health's National Institute on Aging (NIA) Intramural Research Program between 2001 and 2013. Participants included affected persons with a wide range of heritable connective tissue phenotypes, and unaffected family members. Elements include comprehensive history and physical examination, standardized laboratory data, physiologic measures and imaging, standardized patient-reported outcome measures, and an extensive linked biorepository. Read More

Eur Heart J 2019 Jan 30. Epub 2019 Jan 30.

Division of Cardiology, Vancouver General Hospital, 2775 Laurel St, 9th Floor, Vancouver, British Columbia V5Z 1M9, Canada.

Aims: Spontaneous coronary artery dissection (SCAD) was underdiagnosed and poorly understood for decades. It is increasingly recognized as an important cause of myocardial infarction (MI) in women. We aimed to assess the natural history of SCAD, which has not been adequately explored. Read More

Neth Heart J 2019 Jan 25. Epub 2019 Jan 25.

Department of Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands.

Spontaneous coronary artery dissection (SCAD) represents around 25% of cases of acute coronary syndromes (ACS) in women aged 40-65 years who have few or no traditional cardiovascular risk factors. It is assumed that the incidence is underestimated, as the angiographic appearance of SCAD may often mimic atherosclerosis. This review aims to examine SCAD by focusing on the associated predisposing factors and precipitating stressors in this heterogeneous patient population, as well as the best treatment approach and the prognosis. Read More

Am J Cardiol 2019 Apr 10;123(7):1169-1172. Epub 2019 Jan 10.

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

Fibromuscular dysplasia (FMD) is defined by focal narrowing of small and medium-sized arteries due to an idiopathic, noninflammatory, nonatherosclerotic vascular disease. The population-based prevalence of FMD remains unknown. Using the National Inpatient Sample database, we evaluated the prevalence, clinical presentation, mortality, and associated conditions of FMD from January 1, 2004, to September 30, 2015. Read More

Vasc Med 2019 Jan 16:1358863X18821816. Epub 2019 Jan 16.

6 Paris Descartes University, Paris, France.

This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD), which was commissioned by the working group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Read More

BMC Nephrol 2019 Jan 14;20(1):19. Epub 2019 Jan 14.

Section of Nephrology, Department of Medicine, Regina General Hospital, 1440, 14th Avenue, Regina, S4P 0W5, Canada.

Background: We report a 61-year-old female with end-stage renal disease (ESRD) secondary to polycystic kidney disease (PKD) complicated by intradialytic hypertension (IDH). Increased sympathetic drive leading to increased stroke volume and/or vasoconstriction with an inappropriate increase in peripheral vascular resistance (PVR) has been postulated to be the cause of IDH.

Case Presentation: Attempts to control her blood pressure by reducing her goal weight; increasing dialysis times/ frequency and decreasing her sodium concentrate in the dialysis fluid were unsuccessful. Read More

J Hypertens 2019 Feb;37(2):229-252

Paris Descartes University.

: This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD) which was commissioned by the Working Group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Read More

Hypertension 2019 Feb;73(2):371-378

APHP, Hôpital Européen Georges Pompidou, Hypertension Unit, Paris, France (H.K., A.L., M.F., X.J., S.L., P.B., M.A.).

Arterial fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease, whose pathophysiology is still unknown. We performed deep image-based vascular phenotyping of nonaffected arteries to look for systemic vascular alterations in fibromuscular dysplasia. This single center cross-sectional study included 50 patients with multifocal renal fibromuscular dysplasia, 50 hypertensive patients, and 50 healthy controls, matched for age, sex, and ethnicity; hypertensive patients were matched also for blood pressure. Read More

Blood Press 2019 Apr 9;28(2):139-143. Epub 2019 Jan 9.

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

We report the case of a 42-year-old patient referred for suspicion of fibromuscular dysplasia in the context of a carotid artery dissection occurring after a minor trauma. Initial complaints included left hemicrania, lateral diplopia with left 6 cranial nerve palsy and pulsatile tinnitus. The work-up disclosed a large left carotid-cavernous fistula, as well as more proximal carotid lesions compatible with multifocal fibromuscular dysplasia. Read More

J Am Coll Cardiol 2019 Jan;73(1):67-69

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

J Am Coll Cardiol 2019 Jan;73(1):58-66

INSERM, Paris Cardiovascular Research Center, Paris, France; Faculty of Medicine, Paris-Descartes University, Sorbonne Paris Cité, Paris, France. Electronic address:

Background: Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndromes (ACS) afflicting predominantly younger to middle-aged women. Observational studies have reported a high prevalence of extracoronary vascular anomalies, especially fibromuscular dysplasia (FMD) and a low prevalence of coincidental cases of atherosclerosis. PHACTR1/EDN1 is a genetic risk locus for several vascular diseases, including FMD and coronary artery disease, with the putative causal noncoding variant at the rs9349379 locus acting as a potential enhancer for the endothelin-1 (EDN1) gene. Read More

Int Urol Nephrol 2019 Feb 3;51(2):285-291. Epub 2019 Jan 3.

Department of Interventional Radiology and Vascular Surgery, Peking University First Hospital, 8 Xishiku St, Beijing, 100034, People's Republic of China.

Purpose: To evaluate in a retrospective, single-center, case series if percutaneous transluminal renal angioplasty/stenting of chronic renal artery occlusion is beneficial to renal function and blood pressure control, which remains controversial.

Methods: Data from 15 consecutive patients with renal artery stump who underwent successful percutaneous transluminal renal angioplasty/stenting only for unilateral chronic renal artery occlusion at our center from January 2007 to February 2018 and completed follow-up were retrospectively evaluated. Of the 15 patients, 14 (93. Read More

Hypertension 2019 Jan;73(1):7-12

Department of Internal Medicine, Cardiovascular Research Institute Maastricht, Maastricht University Medical Centre, the Netherlands (P.W.d.L., A.A.K.).

Stroke 2018 Dec;49(12):2872-2876

From the Department of Neurology, University of Minnesota Medical School, Minneapolis (A.J.Z., P.D., D.A., J.K., B.J., C.S.).

Background and Purpose- Carotid web (CW) is a rare form of focal fibromuscular dysplasia defined as an abnormal shelf-like projection of intimal fibrous tissue into the carotid bulb. It is theorized that CW leads to ischemic stroke secondary to blood flow stasis and subsequent embolization. The natural history and optimal management of CW are unclear. Read More

Blood Press 2019 02 18;28(1):49-56. 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 nonatherosclerotic 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 11;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 2019 Jan 30;123(2):249-253. 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 2019 Mar 23;93(4):631-634. 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 2019 Mar 2;132(3):329-332. 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 2019 Feb 17;53(2):150-153. 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