Background-We previously described a strong association between fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection. Angiographic manifestations of coronary FMD aside from dissection were considered rare. However, we observed several coronary FMD angiographic abnormalities with corresponding optical coherence tomography abnormalities.
Methods and Results-Baseline demographics and imaging of patients with suspected coronary FMD at Vancouver GeneralHospital 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 irregular stenosis, that is, stenosis with irregular borders in a focal or diffuse pattern with/without systolic accentuation; smooth stenosis, diffuse or focal; segmental dilatation/ectasia; and tortuosity. Optical coherence tomography was performed in a subset of patients. Of 32 patients with extracoronary FMD and suspected coronary involvement, 28 were women (88%), and their mean age was 59.4±9.9 years. Nineteen presented with myocardial infarction (13 caused by spontaneous coronary artery dissection), and 13 had stable symptoms. The observed coronary angiographic abnormalities included tortuosity in all cases (91% were moderate to severe), irregular stenosis in 59%, smooth stenosis in 19%, and segmental dilatation/ectasia in 56%. [11][12][13] However, the angiographic manifestation of CFMD is elusive and not well described. A recent attempt to describe CFMD angiographic features mostly reiterated the appearance of overlying SCAD. 7 Because there are many potential causes of SCAD besides CFMD, the presence of SCAD is not synonymous with CFMD.The angiographic appearance of CFMD in nondissected coronary arteries is reported to be rare and had not been previously studied. Because Vancouver General Hospital is a quaternary referral center for SCAD, a relatively large volume of patients with SCAD and FMD are seen at our cardiac catheterization laboratory. We observed several angiographic abnormalities in nondissected portions of the coronary tree in patients with extracoronary FMD, which we believe to represent CFMD because these features are similar to FMD changes observed in extracoronary beds. We sought to describe and classify these abnormal coronary angiographic appearances of CFMD and to provide the underlying optical coherence tomography (OCT) findings.
MethodsWe performed a retrospective chart and angiographic review of patients with documented FMD in at least 1 extracoronary vascular bed and angiographic nonatherosclerotic coronary artery abnormalities that were suspected to be attributable to CFMD. This cohort included patients who underwent coronary angiography at Vancouver General Hospital for stable symptoms or acute coronary syndrome. We searched for the term FMD in our cardiac catheterization database and our SCAD registries over a 5-year period. These coronary angiograms were rev...