Acute Coronary Syndrome in A 54-Year-Old Female with MoyaMoya Disease: A Case Report

Case Diagnosis: Acute Coronary Syndrome secondary to MoyaMoya Disease

Case Description or Program Description: A 54-year-old Caucasian female with history of ischemic cerebrovascular accident due to MoyaMoya disease with residual right sided hemiparesis, dysphagia and moderate cognitive linguistic deficits was admitted to the inpatient rehabilitation following hospitalization for acute onset chest pain. Electrocardiogram during acute hospitalization was notable for ST segment elevations and she underwent cardiac catheterization with percutaneous coronary intervention (PCI). Following the procedure, she had continued chest pain and underwent repeat catheterization with intravascular ultrasound showing decreased left anterior descending artery luminal area; treated with repeat PCI. Given the lack of significant atherosclerotic build up and heart disease risk factors, her coronary artery disease was attributed to acute stenotic changes from MoyaMoya disease progression.

Setting: Inpatient Rehabilitation Facility

Assessment/Results: Acute Coronary Syndrome Secondary to MoyaMoya Disease. The patient completed a course of inpatient cardiac rehabilitation without complication and was discharged back home to family.

Discussion (relevance): MoyaMoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries with formation of abnormal collateral vasculature. Classically, MoyaMoya disease leads to neurologic pathology including transient ischemic attacks, cerebrovascular accidents, headaches, and neurocognitive impairments. However, systemic vascular complications have also been reported. Notably, up to 4.6% of patients with MoyaMoya disease develop symptomatic coronary artery disease, often with early onset and in the absence of cardiovascular risk factors.

Conclusions: This case highlights the potential for systemic vascular complications in MoyaMoya disease, particularly early onset coronary artery disease in the absence of cardiovascular risk factors; and shows the importance for clinicians to maintain high index of suspicion for extra-cranial complications in patients with MoyaMoya disease.