Daniella Kadian-Dodov1, Heather L Gornik2, Xiaokui Gu3, James Froehlich3, J Michael Bacharach4, Yung-Wei Chi5, Bruce H Gray6, Michael R Jaff7, Esther S H Kim2, Pamela Mace8, Aditya Sharma9, Eva Kline-Rogers2, Christopher White10, Jeffrey W Olin11. 1. 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. 2. Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic Heart and Vascular Institute, Cleveland, Ohio. 3. Cardiovascular Center, University of Michigan, Ann Arbor, Michigan. 4. Department of Cardiology, Avera Heart Hospital of South Dakota, Sioux Falls, South Dakota. 5. Division of Cardiovascular Medicine, University of California Davis Medical Center, Sacremento, California. 6. Vascular Health Alliance, Greenville, South Carolina. 7. Fireman Vascular Center, Massachusetts General Hospital, Boston, Massachusetts. 8. Fibromuscular Dysplasia Society of America, Rocky River, Ohio. 9. Division of Cardiovascular Medicine, University of Virginia, Charlottesville, Virginia. 10. Department of Cardiovascular Diseases, John Ochsner Heart and Vascular Institute, Ochsner Medical Center, University of Queensland Ochsner Clinical School, New Orleans, Louisiana. 11. 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.
Abstract
BACKGROUND: Fibromuscular dysplasia (FMD) is a noninflammatory arterial disease that predominantly affects women. The arterial manifestations may include beading, stenosis, aneurysm, dissection, or tortuosity. OBJECTIVES: This study compared the frequency, location, and outcomes of FMD patients with aneurysm and/or dissection to those of patients without. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND:Fibromuscular dysplasia (FMD) is a noninflammatory arterial disease that predominantly affects women. The arterial manifestations may include beading, stenosis, aneurysm, dissection, or tortuosity. OBJECTIVES: This study compared the frequency, location, and outcomes of FMDpatients with aneurysm and/or dissection to those of patients without. METHODS: The U.S. Registry for FMD involves 12 clinical centers. This analysis included clinical history, diagnostic, and therapeutic procedure results for 921 FMDpatients enrolled in the registry as of October 17, 2014. RESULTS: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. FMDpatients 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 aneurysmpatients (63 of 200) underwent therapeutic intervention for aneurysm repair. CONCLUSIONS: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: Henry D Lather; Heather L Gornik; Jeffrey W Olin; Xiaokui Gu; Steven T Heidt; Esther S H Kim; Daniella Kadian-Dodov; Aditya Sharma; Bruce Gray; Michael R Jaff; Yung-Wei Chi; Pamela Mace; Eva Kline-Rogers; James B Froehlich Journal: JAMA Neurol Date: 2017-09-01 Impact factor: 18.302
Authors: Jeffrey W Olin; Antonio F Di Narzo; Valentina d'Escamard; Daniella Kadian-Dodov; Haoxiang Cheng; Adrien Georges; Annette King; Allison Thomas; Temo Barwari; Katherine C Michelis; Rihab Bouchareb; Emir Bander; Anelechi Anyanwu; Paul Stelzer; Farzan Filsoufi; Sander Florman; Mete Civelek; Stephanie Debette; Xavier Jeunemaitre; Johan L M Björkegren; Manuel Mayr; Nabila Bouatia-Naji; Ke Hao; Jason C Kovacic Journal: Cardiovasc Res Date: 2020-01-01 Impact factor: 10.787
Authors: Imad Bagh; Jeffrey W Olin; James B Froehlich; Eva Kline-Rogers; Bruce Gray; Esther S H Kim; Aditya Sharma; Ido Weinberg; Bryan J Wells; Xiaokui Gu; Heather L Gornik Journal: JAMA Cardiol Date: 2018-08-01 Impact factor: 14.676