埃博斯坦畸形是一种罕见的先天性心脏疾病，主要累及三尖瓣和右室，其主要特征为三尖瓣附着点明显下移，常常合并右室继发改变及合并多种先天畸形，临床变异较大，部分患者可存活至成年。随着对这一疾病的深入认识，特别是基因遗传研究、多模态影像技术以及外科手术水平的提高，在埃博斯坦畸形诊治上近年得到明显的重视。现重点对埃博斯坦畸形诊治上的最新进展做一综述，以加深对埃博斯坦畸形这一罕见疾病的认识。“,”Ebstein anomaly( EA) is one kind of rare congenital heart disease, which is characterized with apical displacement of tri-cuspid value and right ventricular myopathy.Combined cardiac anomaly is not rare and manifestation is variable.More and more EA patients could survive into adulthood.In recent years, researches in molecular genetics, imaging techniques and surgery technique got rapidly devel-oped, and EA attracted more attention as a very challenging disease.This review aims to provide a comprehensive overview of the state of art knowledge in EA.