Abstract
Introduction: Moyamoya disease (MMD) is a rare, progressive steno-occlusive disease of the intracranial carotid arteries. This is primarily a cerebrovascular disease and is mostly seen in young Japanese patients. We report a case of severe stenosis in the distal left circumflex artery (dLCx) in a non-Japanese patient with previously diagnosed MMD. Case presentation: A 33-year-old Caucasian female with history of MMD and 2 CVAs presented with chest pain for 2 days. Her only medication at home was aspirin. There was no history of hypertension, diabetes mellitus, or tobacco use. There was no family history of early CAD. Physical examination was positive for tachycardia but negative for carotid or femoral bruits. Neurologic examination revealed decreased touch sensation and diminished deep tendon reflexes on the left side. ECG demonstrated tachycardia with T wave inversions in leads III and ST depression in lead II. Acute CVA was ruled out with an MRI. Cardiac troponins were minimally elevated with peak of 0.12 ng/dl. An initial diagnosis of NSTEMI was made; patient was loaded with aspirin, clopidogrel and started on a heparin drip. Cardiac catheterization on the following day revealed single vessel CAD in the dLCx, with 80% stenosis and TIMI flow 2. A drug eluting stent was successfully placed in the dLCx. Angiogram post-stent placement showed optimal angiographic result with TIMI 3 flow. The patient was discharged home on dual antiplatelet therapy, statin, beta-blocker and ACE inhibitor. Discussion: MMD is thought to affect the coronary arteries from fibrous intimal thickening and histopathology of these coronary lesions show a homogenous, soft intimal proliferation with minimum lipid deposition and without substantial inflammatory cell infiltration. Although the combined involvement of carotid and coronary artery stenosis is rare, coronary involvement should be considered as one of the causes of ischemic heart disease in young patients with MMD. To the best of our knowledge, this is the first reported case of combined involvement of carotid and coronary artery stenosis in a Caucasian patient with previously diagnosed MMD. It is imperative to educate patients with MMD about possible CAD so that they seek immediate medical attention if cardiac symptoms occur.
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