Yuan Shi-Min, Jing Hua
Department of Cardiothoracic Surgery, Affiliated Hospital of Taishan Medical College, Taian 27100, Shandong Province, People's Republic of China.
Rev Bras Cir Cardiovasc. 2011 Jan-Mar;26(1):107-15. doi: 10.1590/s0102-76382011000100019.
Cystic medial necrosis (CMN) is a disorder of large arteries, in particular the aorta, characterized by an accumulation of basophilic ground substance in the media with cyst-like lesions. CMN is known to occur in certain connective tissue diseases such as Marfan syndrome, Ehlers-Danlos syndrome, and annuloaortic ectasia, which usually result from degenerative changes in the aortic wall. The relationships between CMN and congenital heart defects as well as other disorders have been evidenced. The mechanisms are still controversial, even though many molecular studies have been conducted. The aim of the present article is to provide a comprehensive overview of the CMN lesion in terms of pathologic features, clinical implications and etiologies based on molecular research results.
囊性中层坏死（CMN）是一种大动脉疾病，尤其是主动脉疾病，其特征是中膜出现嗜碱性基质积聚并伴有囊肿样病变。已知CMN发生于某些结缔组织疾病，如马方综合征、埃勒斯-当洛综合征和主动脉环扩张症，这些疾病通常由主动脉壁的退行性改变引起。CMN与先天性心脏缺陷以及其他疾病之间的关系已得到证实。尽管已经进行了许多分子研究，但其机制仍存在争议。本文旨在根据分子研究结果，从病理特征、临床意义和病因方面对CMN病变进行全面概述。
