Fibromuscular Dysplasia
2012; Lippincott Williams & Wilkins; Volume: 125; Issue: 18 Linguagem: Espanhol
10.1161/circulationaha.111.090449
ISSN1524-4539
AutoresStacey Poloskey, Jeffrey W. Olin, Pamela Mace, Heather L. Gornik,
Tópico(s)Cardiovascular and Diving-Related Complications
ResumoHomeCirculationVol. 125, No. 18Fibromuscular Dysplasia Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessBrief ReportPDF/EPUBFibromuscular Dysplasia Stacey L. Poloskey, BS, Jeffrey W. Olin, DO, Pamela Mace, RN and Heather L. Gornik, MD, MHS Stacey L. PoloskeyStacey L. Poloskey From the Cleveland Clinic Heart and Vascular Institute and the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH (S.L.P., H.L.G.); Zena and Michael A. Wiener Cardiovascular Institute and Marie-José and Henry R. Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, NY (J.W.O.); and Fibromuscular Dysplasia Society of America, Rocky River, OH (P.M.). , Jeffrey W. OlinJeffrey W. Olin From the Cleveland Clinic Heart and Vascular Institute and the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH (S.L.P., H.L.G.); Zena and Michael A. Wiener Cardiovascular Institute and Marie-José and Henry R. Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, NY (J.W.O.); and Fibromuscular Dysplasia Society of America, Rocky River, OH (P.M.). , Pamela MacePamela Mace From the Cleveland Clinic Heart and Vascular Institute and the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH (S.L.P., H.L.G.); Zena and Michael A. Wiener Cardiovascular Institute and Marie-José and Henry R. Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, NY (J.W.O.); and Fibromuscular Dysplasia Society of America, Rocky River, OH (P.M.). and Heather L. GornikHeather L. Gornik From the Cleveland Clinic Heart and Vascular Institute and the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH (S.L.P., H.L.G.); Zena and Michael A. Wiener Cardiovascular Institute and Marie-José and Henry R. Kravis Center for Cardiovascular Health, Mount Sinai School of Medicine, New York, NY (J.W.O.); and Fibromuscular Dysplasia Society of America, Rocky River, OH (P.M.). Originally published8 May 2012https://doi.org/10.1161/CIRCULATIONAHA.111.090449Circulation. 2012;125:e636–e639The circulatory system is made up of the heart and blood vessels. There are 3 major types of blood vessels: arteries, veins, and lymphatics. The heart is the pumping organ and helps to push oxygen- and nutrient-rich blood through the arteries to the organs and the limbs. The veins return blood from the organs and limbs to the heart so that it can be resupplied with oxygen from the lungs. The lymphatics connect with the veins and help to return fluid from tissues and skin. This article focuses on a disorder of the arteries known as fibromuscular dysplasia.What Is Fibromuscular Dysplasia?Fibromuscular dysplasia (also known as FMD) is a medical condition characterized by abnormal cell growth in the walls of arteries of the body. FMD is different from other blood vessel disorders that affect the arteries, such as atherosclerosis (blockage of arteries secondary to cholesterol plaque), vasculitis (inflammation of the arteries), and thrombosis (formation of blood clots). FMD can lead to different abnormalities of arteries, such as narrowing, a beaded appearance (Figure, A and B), or even serious complications such as aneurysms (bulging of the arteries) or dissections (tears of the arteries). FMD is not known to involve the veins or the lymphatic system.Download figureDownload PowerPointFigure. Fibromuscular dysplasia in the carotid and renal arteries as imaged with angiography, the gold standard test. A and B, Medial fibroplasia in the internal carotid (A) and renal (B) arteries with the classic string of beads appearance. C and D, The less common intimal fibroplasia, which presents as a focal, bandlike narrowing in the internal carotid (C) and renal (D) arteries.FMD most commonly affects the arteries that supply blood to the kidneys (renal arteries) and brain (carotid and vertebral arteries), but it can occur in almost any artery, including those that supply the intestines (mesenteric arteries), the arms (brachial arteries), and the legs (iliac arteries). In many patients, FMD is found in more than 1 artery.FMD is classified according to the layer of the artery wall that is most involved (Figure). The most common type is medial fibroplasia. Medial fibroplasia affects the middle layer of the artery wall and leads to a very typical beaded appearance of the vessel, which some call a string of beads. Intimal fibroplasia is a less common type that affects the inner layer of the artery wall and leads to an area of smooth narrowing of the vessel.Who Is Affected by FMD?FMD is considered uncommon, but the frequency of this condition in the general population is not known. Recent studies suggest that it is not as rare as initially thought. The majority of patients diagnosed with FMD are female; however, men can also have FMD. Data from the United States FMD patient registry suggest that patients are typically diagnosed with FMD in their early 50s and that there is no significant difference in the age of diagnosis between men and women. A percentage of patients with FMD are diagnosed in childhood. In children, intimal fibroplasia is the most common type.What Causes FMD?The cause of FMD is unknown. A number of theories have been proposed, including environmental factors, such as smoking and estrogen, as well as genetic factors. Approximately 10% of patients with FMD have an affected family member. Understanding the cause(s) of FMD continues to be an area of active investigation.What Are the Symptoms and Signs of FMD?Patients with FMD experience a variety of symptoms that are largely dependent on the location of the affected arteries (Table); however, many patients have no symptoms at all and are diagnosed when an imaging test of the arteries is performed for another reason. Patients with carotid artery FMD may experience headaches (especially migraine type), neck pain, and a pulsatile ringing or swooshing sound in the ears. When patients with carotid FMD are examined, physicians may hear a bruit in the neck (abnormal sound heard when listening over the carotid artery) or may note abnormalities in the pupils of the eyes or the eyelids. The most common sign of renal artery FMD is high blood pressure. In some cases, a physician may hear a bruit when listening over the abdomen or the flanks. Patients with mesenteric (intestinal) FMD can have abdominal pain after eating or weight loss. If FMD involves the arteries of the arms or legs, patients can experience pain with exertion that is relieved by rest, also known as claudication.Table. Signs and Symptoms of FMD, Which Vary Depending on the Blood Vessels That Are InvolvedRenal High blood pressure Bruit heard over the abdomen or flanks Renal artery aneurysm Renal artery dissectionCerebrovascular Headaches (especially migraine type) Pulsatile ringing or swooshing noise in ears Stroke or ministroke (TIA) Neck pain Dizziness Bruit heard over the carotid arteries Brain aneurysm Carotid or vertebral artery dissectionOther sites Abdominal pain after eating (mesenteric FMD) Weight loss (mesenteric FMD) Arm or leg pain with exertion (lower or upper extremity FMD) Femoral bruit (iliac FMD) Heart attack caused by coronary artery dissection (rare)FMD indicates fibromuscular dysplasia; TIA, transient ischemic attack.What Are Warning Signs of FMD?Patients with FMD can develop an aneurysm or dissection in the affected arteries that can cause severe pain. Depending on the artery involved, this may lead to new-onset or worsening headache, neck pain, or abdominal pain. Unfortunately, some patients with carotid artery FMD experience stroke. Patients with FMD and sudden changes in vision or speech, new-onset weakness in an arm or leg, and/or alterations in consciousness should seek immediate medical care.How Is FMD Diagnosed?All patients in whom a diagnosis of FMD is being considered should undergo a detailed medical history and thorough vascular examination; however, a definitive diagnosis of FMD can only be made with imaging studies. Catheter-based angiography is the most accurate imaging technique. During the angiography procedure, a catheter (long slender tube) is inserted into a large artery and slowly advanced until it reaches the vessel of interest. A small amount of contrast is then injected through the catheter, and radiographic (x-ray) pictures of the arteries are taken. The use of contrast material is necessary for the arteries to appear on the radiographic pictures. Catheters may also be used to measure the pressure inside of an artery, which helps to determine the severity of disease. Computed tomography angiography and magnetic resonance angiography are other imaging techniques that may be used to diagnose FMD. These are particularly useful to evaluate arteries in the brain. Doppler ultrasound may be used in both the diagnosis and follow-up of FMD. Note that computed tomography angiography, magnetic resonance angiography, and ultrasound are noninvasive imaging techniques; therefore, no catheter is placed inside the body to image the arteries when these are performed.What Treatment Options Are Available?There is no cure for FMD. Many patients with carotid or vertebral artery involvement will be prescribed aspirin to reduce the risk of stroke. Patients with renal artery FMD often take medications to help control the blood pressure, particularly certain classes of blood pressure medications known as angiotensin-converting enzyme inhibitors or angiotensin receptor blockers.Some patients with FMD may be advised to undergo percutaneous balloon angioplasty. The goal of balloon angioplasty is to widen a narrowing in the affected artery to improve blood flow. Improved blood flow in the renal arteries may result in better blood pressure control, whereas improved blood flow in the carotid arteries may help to prevent stroke. During angioplasty, a balloon on a guidewire is inserted through a catheter (tube) placed in an artery in the groin and advanced to the artery that is narrowed. The balloon is then inflated to open the artery, deflated, and withdrawn.Angioplasty is recommended for patients with renal artery FMD who have uncontrollable blood pressure, intolerance of medications, or declining kidney function. For some patients with newly diagnosed high blood pressure caused by renal artery FMD, angioplasty may also be recommended. Unlike patients with renal artery disease caused by atherosclerosis (or plaque), for patients with renal FMD, balloon angioplasty is generally performed without placement of a metallic stent. Angioplasty is most likely to be effective at controlling or curing high blood pressure in patients who do not have other risk factors associated with high blood pressure (such as obesity or diabetes mellitus) and in those who have had high blood pressure for a short period of time ( 500 patients have consented to participate in the FMD patient registry.Additional ResourcesA list of clinical centers participating in the United States FMD patient registry is available at http://www.fmdsa.org/patient_support/patient_registry (accessed January 10, 2012).DisclosuresDrs Gornik and Olin are volunteer (noncompensated) medical advisory board members to the FMDSA. P. Mace is a paid employee of FMDSA. S. Poloskey reports no conflicts.FootnotesCorrespondence to Heather L. Gornik, MD, MHS, Non-Invasive Vascular Laboratory, Cleveland Clinic Heart and Vascular Institute, 9500 Euclid Ave, Desk J35, Cleveland, OH 44195. E-mail [email protected]org. Previous Back to top Next FiguresReferencesRelatedDetailsCited By Bonacina S, Locatelli M, Mazzoleni V, Pezzini D, Padovani A and Pezzini A (2022) Spontaneous cervical artery dissection and fibromuscular dysplasia: Epidemiologic and biologic evidence of a mutual relationship, Trends in Cardiovascular Medicine, 10.1016/j.tcm.2021.01.006, 32:2, (103-109), Online publication date: 1-Feb-2022. Tsivgoulis G, Safouris A and Alexandrov A (2022) Ultrasonography Stroke, 10.1016/B978-0-323-69424-7.00046-6, (641-659.e8), . 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Mousa A and Gill G (2013) Renal fibromuscular dysplasia, Seminars in Vascular Surgery, 10.1053/j.semvascsurg.2014.06.006, 26:4, (213-218), Online publication date: 1-Dec-2013. Dixon S and Safian R (2013) The Year in Interventional Cardiology, Journal of the American College of Cardiology, 10.1016/j.jacc.2013.01.040, 61:15, (1637-1652), Online publication date: 1-Apr-2013. May 8, 2012Vol 125, Issue 18 Advertisement Article InformationMetrics © 2012 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.090449PMID: 22566353 Originally publishedMay 8, 2012 Keywordsperipheral vascular diseasepatient educationfibromuscular dysplasiaPDF download Advertisement SubjectsAngiographyCardiovascular SurgeryComputerized Tomography (CT)ImagingPercutaneous Coronary InterventionPeripheral Vascular DiseaseUltrasound
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