title: Dissection and Aneurysm in Patients With Fibromuscular Dysplasia: Findings From the U.S. Registry for FMD.
abstract: Fibromuscular dysplasia (FMD) is a noninflammatory arterial disease that predominantly affects women. The arterial manifestations may include beading, stenosis, aneurysm, dissection, or tortuosity.
  
  
  
  This study compared the frequency, location, and outcomes of FMD patients with aneurysm and/or dissection to those of patients without.
  
  
  
  The U.S. Registry for FMD involves 12 clinical centers. This analysis included clinical history, diagnostic, and therapeutic procedure results for 921 FMD patients enrolled in the registry as of October 17, 2014.
  
  
  
  Aneurysm occurred in 200 patients (21.7%) and dissection in 237 patients (25.7%); in total, 384 patients (41.7%) had an aneurysm and/or a dissection by the time of FMD diagnosis. The extracranial carotid, renal, and intracranial arteries were the most common sites of aneurysm; dissection most often occurred in the extracranial carotid, vertebral, renal, and coronary arteries. FMD patients with dissection were younger at presentation (48.4 vs. 53.5 years of age, respectively; p < 0.0001) and experienced more neurological symptoms and other end-organ ischemic events than those without dissection. One-third of aneurysm patients (63 of 200) underwent therapeutic intervention for aneurysm repair.
  
  
  
  Patients with FMD have a high prevalence of aneurysm and/or dissection prior to or at the time of FMD diagnosis. Patients with dissection were more likely to experience ischemic events, and a significant number of patients with dissection or aneurysm underwent therapeutic procedures for these vascular events. Because of the high prevalence and associated morbidity in patients with FMD who have an aneurysm and/or dissection, it is recommended that every patient with FMD undergo one-time cross-sectional imaging from head to pelvis with computed tomographic angiography or magnetic resonance angiography.
authors.affiliation: Zena and Michael A. Wiener Cardiovascular Institute and Marie-Joseé and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Daniella
authors.initials: D
authors.lastname: Kadian-Dodov
authors.affiliation: Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic Heart and Vascular Institute, Cleveland, Ohio.
authors.forename: Heather L
authors.initials: HL
authors.lastname: Gornik
authors.affiliation: Cardiovascular Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: Xiaokui
authors.initials: X
authors.lastname: Gu
authors.affiliation: Cardiovascular Center, University of Michigan, Ann Arbor, Michigan.
authors.forename: James
authors.initials: J
authors.lastname: Froehlich
authors.affiliation: Department of Cardiology, Avera Heart Hospital of South Dakota, Sioux Falls, South Dakota.
authors.forename: J Michael
authors.initials: JM
authors.lastname: Bacharach
authors.affiliation: Division of Cardiovascular Medicine, University of California Davis Medical Center, Sacremento, California.
authors.forename: Yung-Wei
authors.initials: YW
authors.lastname: Chi
authors.affiliation: Vascular Health Alliance, Greenville, South Carolina.
authors.forename: Bruce H
authors.initials: BH
authors.lastname: Gray
authors.affiliation: Fireman Vascular Center, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Jaff
authors.affiliation: Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic Heart and Vascular Institute, Cleveland, Ohio.
authors.forename: Esther S H
authors.initials: ES
authors.lastname: Kim
authors.affiliation: Fibromuscular Dysplasia Society of America, Rocky River, Ohio.
authors.forename: Pamela
authors.initials: P
authors.lastname: Mace
authors.affiliation: Division of Cardiovascular Medicine, University of Virginia, Charlottesville, Virginia.
authors.forename: Aditya
authors.initials: A
authors.lastname: Sharma
authors.affiliation: Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic Heart and Vascular Institute, Cleveland, Ohio.
authors.forename: Eva
authors.initials: E
authors.lastname: Kline-Rogers
authors.affiliation: Department of Cardiovascular Diseases, John Ochsner Heart and Vascular Institute, Ochsner Medical Center, University of Queensland Ochsner Clinical School, New Orleans, Louisiana.
authors.forename: Christopher
authors.initials: C
authors.lastname: White
authors.affiliation: Zena and Michael A. Wiener Cardiovascular Institute and Marie-Joseé and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address: jeffrey.olin@mountsinai.org.
authors.forename: Jeffrey W
authors.initials: JW
authors.lastname: Olin
mesh_terms: D000783:Aneurysm / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D000784:Aortic Dissection / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D000072226:Computed Tomography Angiography; D005260:Female; D005352:Fibromuscular Dysplasia / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D014481:United States / Q000453:epidemiology
keywords: angiography; beading; stenosis; tortuosity