Expanding Clinical Phenotype of Fibromuscular Dysplasia
2017; Lippincott Williams & Wilkins; Volume: 70; Issue: 3 Linguagem: Inglês
10.1161/hypertensionaha.117.09646
ISSN1524-4563
Autores Tópico(s)Cardiovascular Issues in Pregnancy
ResumoHomeHypertensionVol. 70, No. 3Expanding Clinical Phenotype of Fibromuscular Dysplasia Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBExpanding Clinical Phenotype of Fibromuscular Dysplasia Jeffrey W. Olin Jeffrey W. OlinJeffrey W. Olin From the Vascular Medicine and Vascular Diagnostic Laboratory, 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. Originally published17 Jul 2017https://doi.org/10.1161/HYPERTENSIONAHA.117.09646Hypertension. 2017;70:488–489Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: January 1, 2017: Previous Version 1 See related article, pp 652–658Before 2004, fibromuscular dysplasia (FMD) was thought to predominately affect the renal arteries.1 In the early studies, the extracranial carotid and vertebral arteries were involved in only 25% to 30% of patients with FMD.1 However, in a recent report, renal artery involvement occurred in 75.3% of 507 patients, extracranial carotid artery in 73.7% of 476 patients, and vertebral artery in 33.4% of 329 patients.2 There are now >1600 patients enrolled in the US registry, and it seems that the phenotypic presentation is expanding to include not only stenosis (multifocal [string of beads] or focal [a discrete area of a single stenosis]) but also aneurysm, dissection, and arterial tortuosity. Although other studies have reported on the frequency of multivessel FMD,3,4 Plouin et al5 have conducted the first prospective study in which cross-sectional imaging (computed tomographic angiogram [CTA] or magnetic resonance angiogram [MRA]) was performed in every patient enrolled in the ARCADIA FMD registry (Assessment of Renal and Cervical Artery Dysplasia). Patients with renal artery FMD underwent CTA or MRA from the aortic arch to the intracranial vessels, and patients with extracranial cerebrovascular FMD underwent CTA or MRA from the diaphragm to the pelvis. In the ARCADIA FMD registry, at least 2 vascular beds were affected by dysplastic stenosis in 48% of participants. However, if a patient had stenosis in 1 vascular bed and either aneurysm or dissection in another vascular bed, they were coded as having single vascular bed involvement. If the patients with aneurysms and dissections were included as having FMD in those vascular beds, then 66.1% of their patients would have multivessel involvement.5 The ARCADIA investigators concluded that renal artery imaging should be considered in hypertensive patients with extracranial cerebrovascular FMD, and cervical artery imaging should be considered in patients with a renal artery presentation and bilateral renal artery stenosis.5Based on the data from the ARCADIA FMD registry and from the US registry for FMD, it is reasonable to go a step further and perform 1-time head to pelvis imaging in all patients with FMD even in the absence of hypertension or bilateral renal artery involvement. We concur with the authors that CTA is the preferred method of imaging because of the higher spacial resolution and thus greater sensitivity and specificity than with MRA.6 Although it is possible to perform head to pelvis imaging at 1 time (using 2 injections because blood is flowing in opposite directions), our standard practice is to perform a CTA of neck and head on 1 occasion and a CTA of the chest, abdomen, and pelvis ≈1 week later. In the US registry, among 921 patients, 200 patients (21.7%) had ≥1 aneurysm, 237 patients (25.7%) had ≥1 dissection, and 53 patients had both a dissection and aneurysm (5.8%).7 Of the 921 patients, 41.7% had an aneurysm and/or dissection (Figure). We are in agreement with the authors that if a patient only presents with an aneurysm or dissection without an arterial stenosis, they should not be labeled as having FMD. However, there is a fundamental difference in the way multisite FMD is classified in the in the US registry as compared with the ARCADIA registry. If a patient has multifocal or focal FMD in 1 arterial bed and an aneurysm, dissection, or tortuosity in another arterial bed, we would classify that patient as having multiple vascular bed involvement. The performance of 1-time head to pelvis imaging is not so much to identify FMD in other vascular territories but to identify potentially serious aneurysms or dissections and either treat or put into a surveillance program of close observation.Download figureDownload PowerPointFigure. United States registry for fibromuscular dysplasia rates of aneurysm and dissection in 921 patients. Reprinted from Kadian-Dodov et al7 with permission of the publisher. Copyright © 2016, The American College of Cardiology Foundation.As the authors' note, the most important limitation of this study (and the US registry for FMD) is that a core laboratory was not used in every patient. Thus, the diagnosis may be missed, leading to an underestimation of the true prevalence of multivessel FMD or FMD inaccurately diagnosed when a less experienced clinician reviews the images. This issue is highlighted in the CORAL trial (Cardiovascular Outcomes in Renal Atherosclerotic Lesions).8 This was a trial comparing renal artery stenting to maximum medical therapy for patients with atherosclerotic renal artery stenosis.8 Patients were randomized to either renal artery stenting and maximal medical therapy or maximal medical therapy alone. After randomization, the images were sent to a core laboratory to over-read. FMD was an exclusion for entry into CORAL, yet 44 of 510 women (8.6%) and 14 of 505 men (2.8%) had a core laboratory diagnosis of FMD indicating underdiagnosis of FMD.8 The goal for all future FMD reports should be to use a core laboratory made of up individuals with special expertise in FMD who are blinded to the other characteristics of the patient population.Another limitation of the current study is that spontaneous coronary artery dissection (SCAD) was not present in any patient in the current report. The association of SCAD and FMD was not known at the onset of the ARCADIA registry. Multiple series have now shown that FMD is a common finding in patients who present with SCAD.9–12 Saw et al13 found FMD in 121 of 168 (72%) patients with SCAD. In the Mayo Clinic SCAD registry, 79 of 112 (70%) patients with SCAD had extracoronary vascular involvement, and of these, FMD was the most common vascular abnormality occurring in 80% of subjects.14 In the US registry, among the 237 patients with dissection, 25 (10.5% of all dissection patients) had coronary artery dissection.7Another interesting arterial finding, not commented on in the paper from the ARCADIA registry, is the increased frequency of extreme cervical and coronary tortuosity in patients with FMD.9,12,15 We showed that 37 (32%) of 116 patients with FMD had extreme tortuosity of the mid- and distal internal carotid artery, which we termed the S curve.15 For each FMD patient with an S curve, there were 2 age- and sex-matched controls and 2 controls over the age of 70 years. Among the 74 age and sex controls, there were only 2 patients with S curves (2.7%), and among 74 controls above the age of >70 years, there were 12 (16.2%) patients with S curves. Although the S curve is not specific for FMD (may occur in other diseases associated with arterial tortuosity, such as Loeys–Dietz syndrome, Ehlers-Danlos syndrome type IV, and arterial tortuosity syndrome), it occurs in ≈1/3 of patients with FMD, and its presence in a patient below the age of 70 years should prompt the clinician to consider the diagnosis of FMD.Plouin et al5 have made an important contribution to the understanding of FMD, namely that this disease is not an isolated condition of 1 vascular territory but a systemic disease affecting multiple vascular territories and associated with not only stenosis of an artery but also aneurysms and dissections. They have provided compelling evidence that every patient with FMD should undergo 1-time screening with CTA or MRA from the head to the pelvis.DisclosuresJ.W. Olin served as Chair, Medical Advisory Board, Fibromuscular Dysplasia Society of America Volunteer position.FootnotesThe opinions expressed in this editorial are not necessarily those of the editors or of the American Heart Association.Correspondence to Jeffrey W. Olin, Vascular Medicine and Vascular Diagnostic Laboratory, 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, One Gustave L. Levy Pl, Box 1033, New York, NY 10029. E-mail [email protected]References1. Slovut DP, Olin JW. Fibromuscular dysplasia.N Engl J Med. 2004; 350:1862–1871. doi: 10.1056/NEJMra032393.CrossrefMedlineGoogle Scholar2. Kim ES, Olin JW, Froehlich JB, Gu X, Bacharach JM, Gray BH, Jaff MR, Katzen BT, Kline-Rogers E, Mace PD, Matsumoto AH, McBane RD, White CJ, Gornik HL. 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The S curve: a novel morphological finding in the internal carotid artery in patients with fibromuscular dysplasia.Vasc Med. 2014; 19:356–362. doi: 10.1177/1358863X14547122.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited BySchwartz A, Kim E, Gleason P, Li X, Ko Y and Wells B (2022) Aortic Dimensions Are Larger in Patients With Fibromuscular Dysplasia, Journal of the American Heart Association, 11:12, Online publication date: 21-Jun-2022. Persu A, Dobrowolski P, Gornik H, Olin J, Adlam D, Azizi M, Boutouyrie P, Bruno R, Boulanger M, Demoulin J, Ganesh S, J. Guzik T, Januszewicz M, Kovacic J, Kruk M, de Leeuw P, Loeys B, Pappaccogli M, Perik M, Touzé E, Van der Niepen P, Van Twist D, Warchoł-Celińska E, Prejbisz A and Januszewicz A (2021) Current progress in clinical, molecular, and genetic aspects of adult fibromuscular dysplasia, Cardiovascular Research, 10.1093/cvr/cvab086, 118:1, (65-83), Online publication date: 7-Jan-2022. 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Ciurică S, Lopez-Sublet M, Loeys B, Radhouani I, Natarajan N, Vikkula M, Maas A, Adlam D and Persu A (2019) Arterial Tortuosity, Hypertension, 73:5, (951-960), Online publication date: 1-May-2019. Gilhofer T and Saw J (2019) Spontaneous coronary artery dissection: a review of complications and management strategies, Expert Review of Cardiovascular Therapy, 10.1080/14779072.2019.1598261, 17:4, (275-291), Online publication date: 3-Apr-2019. Kadian-Dodov D (2019) Fibromuscular dysplasia: Beginning to see the forest through the trees, Vascular Medicine, 10.1177/1358863X19826346, 24:2, (120-121), Online publication date: 1-Apr-2019. Van der Niepen P, van Tussenbroek F, Devos H, Debing E, Di Monaco S, Goffette P, Astarci P and Persu A (2018) Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency, European Journal of Clinical Investigation, 10.1111/eci.13023, 48:11, (e13023), Online publication date: 1-Nov-2018. Hayes S, Kim E, Saw J, Adlam D, Arslanian-Engoren C, Economy K, Ganesh S, Gulati R, Lindsay M, Mieres J, Naderi S, Shah S, Thaler D, Tweet M and Wood M (2018) Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association, Circulation, 137:19, (e523-e557), Online publication date: 8-May-2018. Narula N, Kadian-Dodov D and Olin J (2018) Fibromuscular Dysplasia: Contemporary Concepts and Future Directions, Progress in Cardiovascular Diseases, 10.1016/j.pcad.2018.03.001, 60:6, (580-585), Online publication date: 1-Mar-2018. September 2017Vol 70, Issue 3 Advertisement Article InformationMetrics © 2017 American Heart Association, Inc.https://doi.org/10.1161/HYPERTENSIONAHA.117.09646PMID: 28716991 Originally publishedJuly 17, 2017 PDF download Advertisement SubjectsHypertensionVascular Disease
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