Angiographic and Intracoronary Manifestations of Coronary Fibromuscular Dysplasia
Background—We previously described a strong association between fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection (SCAD). Angiographic manifestations of coronary FMD (CFMD) aside from dissection were considered rare. However, we observed several CFMD angiographic abnormalities with corresponding optical coherence tomography (OCT) abnormalities.
Methods and Results—Baseline demographics and imaging of patients with suspected CFMD at Vancouver General Hospital were reviewed. Presence of multifocal (string-of-beads) extracoronary FMD was confirmed by 2 specialists. In these patients, coronary angiographic findings (excluding dissected segments) were reviewed and classified by 2 experienced angiographers for: (1) irregular stenosis -stenosis with irregular borders in a focal or diffuse pattern and with/without systolic accentuation, (2) smooth stenosis - diffuse or focal, (3) segmental dilatation/ectasia, and (4) tortuosity. OCT was performed in a subset of patients. Of 32 patients with extracoronary FMD and suspected coronary involvement, 28 were women (88%), mean age was 59.4±9.9yrs. Nineteen presented with myocardial infarction (13 due to SCAD), and 13 had stable symptoms. The observed coronary angiographic abnormalities included: tortuosity in all cases (91% were moderate-severe), irregular stenosis in 59%, smooth stenosis in 19%, and segmental dilatation/ectasia in 56%. Fifteen patients had OCT of the abnormal segments showing abnormalities including multiple areas of patchy or diffuse intimal, medial or adventitial abnormalities with thickening/accumulation of varied reflectivities, macrophage infiltration, loss/duplication of elastic membranes, and cavitation.
Conclusions—This is the first case series describing findings suggestive of angiographic and intracoronary manifestations of CFMD. Future studies should prospectively review these features in patients with extracoronary FMD.
- Received November 4, 2015.
- Revision received February 9, 2016.
- Accepted March 3, 2016.