Livesay James, Johnson Jeffrey
Department of Medicine, University of Tennessee Graduate School of Medicine, Knoxville, TN, USA.
Case Rep Cardiol. 2019 Feb 4;2019:7124072. doi: 10.1155/2019/7124072. eCollection 2019.
Moyamoya disease is a rare condition that is primarily reported in Asian populations, characterized by stenoocclusive intracranial angiopathy with small, fragile, and multiple collateral vessel formation. Extracranial complications, mainly abnormalities within the renal vasculature, have been described; however, there are very few case reports of cardiovascular complications in patients with Moyamoya disease. We report a 26-year-old Caucasian female with known Moyamoya disease who presented with both typical and atypical chest pain, mimicking symptoms of a previous non-ST-elevation myocardial infarction. Approximately six months prior to the current hospital admission, she underwent coronary angiography requiring percutaneous coronary intervention (PCI) with two drug-eluting stents to the right coronary artery (RCA) for a critical stenosis. Despite medical management, our patient developed inferior lead ST-elevations leading to a repeat left heart catheterization which showed clinically significant stenosis of the first obtuse marginal branch. Development of significant coronary artery stenosis in a short period of time demonstrates the clinical significance of minimal atherosclerosis in the setting of underlying fibrocellular thickening as seen in patients with Moyamoya disease. Clinicians need to be aware of the possibility of coronary involvement in addition to intracranial vascular complications in patients with Moyamoya disease and take appropriate measures to prevent or delay the development of atherosclerosis in these arteries.
烟雾病是一种主要在亚洲人群中报道的罕见疾病，其特征是颅内血管出现狭窄闭塞性病变，并伴有细小、脆弱且多发的侧支血管形成。已经描述了颅外并发症，主要是肾血管系统内的异常情况；然而，烟雾病患者发生心血管并发症的病例报告非常少。我们报告了一名26岁的白种女性，已知患有烟雾病，她出现了典型和非典型胸痛，类似之前非ST段抬高型心肌梗死的症状。在本次入院前约六个月，她接受了冠状动脉造影，因严重狭窄需要对右冠状动脉（RCA）进行经皮冠状动脉介入治疗（PCI）并植入两枚药物洗脱支架。尽管进行了药物治疗，我们的患者仍出现下壁导联ST段抬高，导致再次进行左心导管检查，结果显示第一钝缘支存在具有临床意义的狭窄。在短时间内出现显著的冠状动脉狭窄，表明在烟雾病患者中，在潜在的纤维细胞增厚背景下，轻微动脉粥样硬化具有临床意义。临床医生需要意识到烟雾病患者除了颅内血管并发症外，还可能存在冠状动脉受累情况，并采取适当措施预防或延缓这些动脉粥样硬化的发展。
