Dissection and Aneurysm in Patients With Fibromuscular Dysplasia: Findings From the U.S. Registry for FMD.
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.
['Kadian-Dodov|Daniella|D|', 'Gornik|Heather L|HL|', 'Gu|Xiaokui|X|', 'Froehlich|James|J|', 'Bacharach|J Michael|JM|', 'Chi|Yung-Wei|YW|', 'Gray|Bruce H|BH|', 'Jaff|Michael R|MR|', 'Kim|Esther S H|ES|', 'Mace|Pamela|P|', 'Sharma|Aditya|A|', 'Kline-Rogers|Eva|E|', 'White|Christopher|C|', 'Olin|Jeffrey W|JW|']
D000783:Aneurysm D000784:Aneurysm, Dissecting D000072226:Computed Tomography Angiography D005260:Female D005352:Fibromuscular Dysplasia D006801:Humans D018810:Magnetic Resonance Angiography D008297:Male D008875:Middle Aged D015995:Prevalence D012042:Registries D012189:Retrospective Studies D012307:Risk Factors D014481:United States
beading stenosis tortuosity angiography