Challenging Case Sample

Recurrent Spontaneous Coronary Artery Dissection in a Patient With Fibromuscular Dysplasia

Patient Initials or Identifier Number: KW

Relevant History and Physical Exam: A 39 year old African American woman with no significant past medical history presented with sudden onset chest pain, nausea and diaphoreses while driving to work. Her physical examination was unremarkable. Relevant Test Results Prior to Catheterization: The patient’s electrocardiogram revealed anterior ST elevations (V1-4). Bedside echocardiogram revealed an ejection fraction of 50%, with mild apical hypokinesis.

Relevant Catheterization Findings: Emergent cardiac catheterization revealed normal RCA and LCx, with a diffuse 75% mid-distal LAD narrowing. IVUS confirmed a dissection in the segment corresponding to the area of angiographic narrowing.

Interventional Management: A sirolimus eluting stents (Cypher RX), 2.5 x 28mm and 2.5 x 18mm were deployed in the distal LAD and the mid LAD respectively. Following second stent deployment dissection propagated proximally requiring additional Cypher RX 3.0 x 13mm stent placement to contain the dissection. IVUS was then used to ensure containment of the dissection and adequate stent strut apposition. The patient was discharged 2 days post-procedure on dual antiplatelet therapy in stable condition. The patient discontinued both aspirin and clopidogrel in spite of medical advice 3 years after initial presentation. Eight years after presentation she developed recurrent chest pain and ruled in for NSTEMI. Cardiac angiogram revealed a dissection in the distal, small sized LCX segment not amenable to interventional treatment. Patient received aspirin and clopidogrel as well as guideline directed medical therapy (GDMT). While on antiplatelet and GDMT an angiography 1 year after LCx dissection revealed a healed dissection and patent stents in the LAD. Magnetic resonance angiography (MRA) revealed a ‘beaded’ appearing right renal artery, most consistent with “multifocal” form of fibromuscular dysplasia (FMD).

Case Description
We describe a case of a healthy African American woman who presents with anterior STEMI secondary to spontaneous coronary artery dissection (SCAD) of LAD. The patient underwent percutaneous intervention, with containment of dissection. Eight years later patient had a recurrent coronary artery dissection in LCx artery when off antiplatelet therapy. She was treated conservatively with dual antiplatelet therapy. Right renal artery ‘beaded’ appearance on magnetic resonance angiography confirmed diagnosis of fibromuscular dysplasia. SCAD is an infrequent cause of ST elevation myocardial infarction with fibromuscular dysplasia being the most common predisposing etiology, especially among middle age women. Treatment options favor conservative approach in stable patients and percutaneous intervention or CABG in patients with active coronary ischemia. Our patient case demonstrates need for tailored approach to particular variant of SCAD presentation in patients with FMD. In addition, it emphasizes need for lifelong aspirin therapy in order to minimize risk of recurrent vascular events associated with FMD.

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