Dissection and Aneurysm in Fibromuscular Dysplasia
What are the frequency, location, and outcomes for fibromuscular dysplasia (FMD) patients with aneurysm and/or dissection, as compared to those 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 and dissection location were recorded in the patient’s past medical history and presenting signs/symptoms, although not all vascular beds were imaged in all patients. Chi-square and Fisher exact tests were performed for comparison.
Aneurysm occurred in 200 (21.7%) and dissection in 237 (25.7%) patients; in total, 384 (41.7%) patients had an aneurysm and/or a dissection. 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; p < 0.0001) and experienced more neurologic symptoms and other end-organ ischemic events compared to those without dissection. One-third (63 of 200) of aneurysm patients underwent a therapeutic intervention for aneurysm repair.
The authors concluded that patients with FMD have a high prevalence of aneurysm and/or dissection prior to or at the time of FMD diagnosis.
This study reports a high prevalence of aneurysm and dissection among patients with FMD. Aneurysm was most often identified in the carotid, renal, and intracranial arteries and was associated with higher rates of therapeutic intervention to repair the aneurysm. These findings support the recommendation of one-time cross-sectional imaging (computed tomography angiography or magnetic resonance angiography) from head to pelvis for all patients with FMD. Additional research is indicated to uncover the genetic and predisposing factors for vascular events in FMD, as well as to define treatment and long-term management strategies for patients with FMD.
< Back to Listings