Division of Cardiovascular Medicine, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN (E.S.H.K.).
Division of Cardiology, Vancouver General Hospital, University of British Columbia Canada (J.S.).
Circ Res. 2021 Jun 11;128(12):1958-1972. doi: 10.1161/CIRCRESAHA.121.318300. Epub 2021 Jun 10.
Multifocal fibromuscular dysplasia (FMD) and spontaneous coronary artery dissection are both sex-biased diseases disproportionately affecting women over men in a 9:1 ratio. Traditionally known in the context of renovascular hypertension, recent advances in knowledge about FMD have demonstrated that FMD is a systemic arteriopathy presenting as arterial stenosis, aneurysm, and dissection in virtually any arterial bed. FMD is also characterized by major cardiovascular presentations including hypertension, stroke, and myocardial infarction. Similar to FMD, spontaneous coronary artery dissection is associated with a high prevalence of extracoronary vascular abnormalities, including FMD, aneurysm, and extracoronary dissection, and recent studies have also found genetic associations between the two diseases. This review will summarize the relationship between FMD and spontaneous coronary artery dissection with a focus on common clinical associations, histopathologic mechanisms, genetic susceptibilities, and the biology of these diseases. The current status of disease models and critical future research directions will also be addressed.
多灶性纤维肌发育不良(FMD)和自发性冠状动脉夹层都是性别偏倚性疾病,女性发病率明显高于男性,比例为 9:1。在肾血管性高血压的背景下,FMD 的知识近年来取得了进展,表明 FMD 是一种系统性动脉疾病,可表现为几乎任何动脉床的动脉狭窄、动脉瘤和夹层。FMD 还以主要心血管表现为特征,包括高血压、中风和心肌梗死。与 FMD 类似,自发性冠状动脉夹层与冠状动脉外血管异常的高患病率相关,包括 FMD、动脉瘤和冠状动脉外夹层,最近的研究还发现这两种疾病之间存在遗传关联。本综述将总结 FMD 和自发性冠状动脉夹层之间的关系,重点关注常见的临床关联、组织病理学机制、遗传易感性以及这些疾病的生物学。还将讨论疾病模型的现状和关键的未来研究方向。