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Published on: March 8, 2019
Aortic Dimensions Are Larger in Patients With Fibromuscular Dysplasia
Arielle M Schwartz1, Esther Kim2, Patrick Gleason3
1Willis Hurst Internal Medicine Residency Program Emory University Atlanta GA.
Abstract:
Background Fibromuscular dysplasia (FMD) is a disease of unknown etiology that causes stenosis, aneurysmal dilatation, and dissection of vascular beds. Known to affect medium-sized arteries, FMD is not typically considered to affect the aorta. We tested the hypothesis that aortic size in FMD is abnormal compared with age- and sex-matched controls. Methods and Results Medical records and computed tomography angiography images were reviewed in female patients with a diagnosis of FMD who were seen in the vascular medicine clinic at Emory Healthcare. Aortic dimensions were measured at 6 different landmarks. Using 2 sample t tests, the aortic measurements and height-indexed measurements were compared with published normal values in healthy women of a similar age. A total of 94 female patients were included in the study. The median age was 57 (interquartile range, 50-65). FMD involvement was present most commonly in the extracranial carotid (77.7%) and renal (43.6%) arteries. All 6 aortic segments were found to be larger in both absolute measures and height-indexed measures in the FMD population (P<0.001). The largest differences were observed within the absolute measures of the sinotubular junction with mean±SD (mm) (29.9±4.1) versus (27±2.5), ascending aorta (32.7±4.4) versus (30.0±3.5), and descending aorta (24.7±3.0) versus (22.0±2.0) (P<0.001). Conclusions Aortic diameters in female patients with FMD are larger when compared with published age- and sex-matched normal values. These findings suggest that FMD may also affect the large-sized arteries.
Insights
Fibromuscular dysplasia (FMD) is a vascular condition that can affect the aorta. This study found that women with FMD have larger aortic dimensions compared to healthy individuals, suggesting FMD may impact large arteries.
Area of Science:
- Vascular Medicine
- Cardiovascular Imaging
- Genetics and Disease Etiology
Background:
- Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vasculopathy of unknown cause.
- It typically affects medium-sized arteries, leading to stenosis, aneurysm, or dissection.
- Aortic involvement in FMD is not widely recognized, challenging current understanding of the disease.
Purpose of the Study:
- To investigate whether aortic dimensions are abnormal in patients diagnosed with FMD.
- To test the hypothesis that aortic size differs in FMD patients compared to age- and sex-matched controls.
Main Methods:
- Retrospective review of medical records and CT angiography images from 94 female FMD patients.
- Measurement of aortic dimensions at six distinct landmarks.
- Comparison of patient aortic measurements with published normative values for healthy women using t-tests.
Main Results:
- Female patients with FMD exhibited significantly larger aortic dimensions, both absolute and height-indexed, across all six measured segments (P<0.001).
- The most pronounced differences were observed in the sinotubular junction, ascending aorta, and descending aorta.
- Common FMD locations included extracranial carotid (77.7%) and renal (43.6%) arteries.
Conclusions:
- Female patients with FMD demonstrate enlarged aortic diameters relative to normative data.
- These findings indicate that FMD may extend its pathological effects to involve large arteries, including the aorta.
- This suggests a broader spectrum of vascular involvement in FMD than previously understood.
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