Dissection and aneurysm in patients with fibromuscular dysplasia findings from the US registry for FMD

Kadian-Dodov, Daniella, Gornik, Heather L., Gu, Xiaokui, Froehlich, James, Bacharach, J. Michael, Chi, Yung-Wei, Gray, Bruce H., Jaff, Michael R., Kim, Esther S. H., Mace, Pamela, Sharma, Aditya, Kline-Rogers, Eva, White, Christopher and Olin, Jeffrey W. (2016) Dissection and aneurysm in patients with fibromuscular dysplasia findings from the US registry for FMD. Journal of the American College of Cardiology, 68 2: 176-185. doi:10.1016/j.jacc.2016.04.044


Author Kadian-Dodov, Daniella
Gornik, Heather L.
Gu, Xiaokui
Froehlich, James
Bacharach, J. Michael
Chi, Yung-Wei
Gray, Bruce H.
Jaff, Michael R.
Kim, Esther S. H.
Mace, Pamela
Sharma, Aditya
Kline-Rogers, Eva
White, Christopher
Olin, Jeffrey W.
Title Dissection and aneurysm in patients with fibromuscular dysplasia findings from the US registry for FMD
Journal name Journal of the American College of Cardiology   Check publisher's open access policy
ISSN 0735-1097
1558-3597
Publication date 2016-07-12
Sub-type Article (original research)
DOI 10.1016/j.jacc.2016.04.044
Open Access Status Not yet assessed
Volume 68
Issue 2
Start page 176
End page 185
Total pages 10
Place of publication San Diego, CA United States
Publisher Elsevier
Collection year 2017
Language eng
Formatted 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 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.
Keyword Angiography
Beading
Stenosis
Tortuosity
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
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