Spontaneous Coronary Artery Dissection in Patients With Fibromuscular Dysplasia

Young women with acute coronary syndrome (ACS) frequently have nonatherosclerotic coronary artery disease (NACAD) and may be misdiagnosed. Coronary fibromuscular dysplasia (CFMD) commonly is overlooked, as the angiographic appearance is often subtle. Our group previously described CFMD as a diffuse obliterative disease starting abruptly at the mid-distal vessel, involving long segments.1 Spontaneous coronary dissection (SCAD) is another common form of NACAD and may be superimposed on CFMD, causing ACS. We report the first case series of SCAD and concomitant fibromuscular dysplasia (FMD). Case 1 (Figure 1)Figure 1. Case 1. A Coronary angiogram of the right coronary artery (RCA) showing diffuse stenosis from the mid-segment to the posterior descending artery. B Optical coherence tomography (OCT) image of the normal proximal RCA (* corresponding to A ). C OCT image showing intramural hematoma in the distal RCA (+ corresponding to A ). D Carotid angiogram showing shelf-like fibromuscular dysplasia (FMD) lesion at the ostium of the left internal carotid artery (*), followed by mild irregularities in the proximal left carotid artery. E Iliac angiogram showing diffuse mild FMD in the left external iliac artery (*).A 35-year-old African female with no cardiovascular (CV) risk factors presented on April 18, 2011 with persistent chest pain for 2 days while doing aerobic exercises. ECG showed T-inversion anteriorly and inferiorly, and troponin I (Tn-I) peaked at 4.2μmol/L. Coronary angiogram showed diffuse severe stenosis from mid-right coronary artery (RCA) to the distal posterior descending artery with TIMI3 flow. A subsequent optical coherence tomography (OCT) showed coronary dissection with extensive intramural hematoma, and she was treated with stents. She had incidental bilateral external iliac artery FMD, and her carotid computer tomography angiography (CTA) showed a flap at the origin of the left carotid artery, prompting catheterization that confirmed FMD of both carotid …