Abstract

Background and Purpose: Coronary artery disease in moyamoya disease (MMD) have been described sporadically in several case reports. The purpose of this study is to determine the prevalence and characteristics of coronary artery disease in patients with MMD. Methods: From August 1991 to December 2012, 446 patients diagnosed with adult MMD at our hospital. Baseline characteristics and prevalence of coronary artery disease were reviewed based on medical records and laboratory findings. The findings of conventional coronary angiography and/or coronary computed tomography were also reviewed for the presence and appearance of coronary artery lesion. Results: - Of 446 patients with adult MMD, 21 patients were found to have coronary artery disease. Ten patients were treated with coronary artery bypass graft (n=4) or percutaneous coronary intervention (n=6) for unstable angina or myocardial infarction. Eleven were treated with medication for stable angina (n=6) and variant angina with mild degree of stenosis (n=5). Median age at diagnosed with coronary artery disease of these patients were 44 (range, 27-59). Two patients showed calcification on coronary artery lesion. Comorbid stroke risk factor rate were 19%, 38%, 9.5% and 19 % in diabetes, hypertension, dyslipidemia and smoking. Six of 21 patients had more than 2 risk factor. Conclusion: - Twenty one (4.7%) of our adult MMD registry patients showed coronary artery disease. And only 2 (9.5%) showed calcification which might means that atherosclerosis burden is low in coronary artery disease with MMD. Coronary artery disease might be a clinically relevant systemic manifestation in patients with MMD, considering early onset coronary diseaes and low prevalence of stroke risk factor.

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