title: Angiographic and Intracoronary Manifestations of Coronary Fibromuscular Dysplasia.
abstract: We previously described a strong association between fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection. Angiographic manifestations of coronary FMD aside from dissection were considered rare. However, we observed several coronary FMD angiographic abnormalities with corresponding optical coherence tomography abnormalities.
  
  
  
  Baseline demographics and imaging of patients with suspected coronary FMD at Vancouver General Hospital were reviewed. Presence of multifocal (string-of-beads) extracoronary FMD was confirmed by 2 specialists. In these patients, coronary angiographic findings (excluding dissected segments) were reviewed and classified by 2 experienced angiographers for irregular stenosis, that is, stenosis with irregular borders in a focal or diffuse pattern with/without systolic accentuation; smooth stenosis, diffuse or focal; segmental dilatation/ectasia; and tortuosity. Optical coherence tomography was performed in a subset of patients. Of 32 patients with extracoronary FMD and suspected coronary involvement, 28 were women (88%), and their mean age was 59.4±9.9 years. Nineteen presented with myocardial infarction (13 caused by spontaneous coronary artery dissection), and 13 had stable symptoms. The observed coronary angiographic abnormalities included tortuosity in all cases (91% were moderate to severe), irregular stenosis in 59%, smooth stenosis in 19%, and segmental dilatation/ectasia in 56%. Fifteen patients had optical coherence tomography of the abnormal segments showing abnormalities, including multiple areas of patchy or diffuse intimal, medial or adventitial abnormalities with thickening/accumulation of varied reflectivities, macrophage infiltration, loss/duplication of elastic membranes, and cavitation.
  
  
  
  This is the first case series describing findings suggestive of angiographic and intracoronary manifestations of coronary FMD. Future studies should prospectively review these features in patients with extracoronary FMD.
authors.affiliation: From Divisions of Cardiology (J.S., J.M.) and Radiology (L.M.), Vancouver General Hospital, BC, Canada; Division of Cardiology, Harrington Heart and Vascular Institute, University Hospital Case Medical Center, Cleveland, OH (H.B.); and Heart and Vascular Institute, Cleveland Clinic Foundation, OH (H.L.G.). jsaw@mail.ubc.ca.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Saw
authors.affiliation: From Divisions of Cardiology (J.S., J.M.) and Radiology (L.M.), Vancouver General Hospital, BC, Canada; Division of Cardiology, Harrington Heart and Vascular Institute, University Hospital Case Medical Center, Cleveland, OH (H.B.); and Heart and Vascular Institute, Cleveland Clinic Foundation, OH (H.L.G.).
authors.forename: Hiram
authors.initials: H
authors.lastname: Bezerra
authors.affiliation: From Divisions of Cardiology (J.S., J.M.) and Radiology (L.M.), Vancouver General Hospital, BC, Canada; Division of Cardiology, Harrington Heart and Vascular Institute, University Hospital Case Medical Center, Cleveland, OH (H.B.); and Heart and Vascular Institute, Cleveland Clinic Foundation, OH (H.L.G.).
authors.forename: Heather L
authors.initials: HL
authors.lastname: Gornik
authors.affiliation: From Divisions of Cardiology (J.S., J.M.) and Radiology (L.M.), Vancouver General Hospital, BC, Canada; Division of Cardiology, Harrington Heart and Vascular Institute, University Hospital Case Medical Center, Cleveland, OH (H.B.); and Heart and Vascular Institute, Cleveland Clinic Foundation, OH (H.L.G.).
authors.forename: Lindsay
authors.initials: L
authors.lastname: Machan
authors.affiliation: From Divisions of Cardiology (J.S., J.M.) and Radiology (L.M.), Vancouver General Hospital, BC, Canada; Division of Cardiology, Harrington Heart and Vascular Institute, University Hospital Case Medical Center, Cleveland, OH (H.B.); and Heart and Vascular Institute, Cleveland Clinic Foundation, OH (H.L.G.).
authors.forename: G B John
authors.initials: GB
authors.lastname: Mancini
mesh_terms: D000368:Aged; D017023:Coronary Angiography / Q000379:methods; D003331:Coronary Vessels / Q000000981:diagnostic imaging*; D005260:Female; D005352:Fibromuscular Dysplasia / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
keywords: coronary angiography; coronary artery disease; fibromuscular dysplasia