2022 年第 11 期 第 17 卷

三尖瓣下移畸形认识与治疗研究进展

Research progress on recognition and treatment of Ebstein′s anomaly

作者：刘畅1王粮山2谌琦1戴庆箐1

英文作者：Liu Chang1 Wang Liangshan2 Chen Qi1 Dai Qingqing1

单位：1清华大学附属北京清华长庚医院老年医学科 清华大学临床医学院，北京102218；2首都医科大学附属北京安贞医院心外危重症中心，北京100029

英文单位：1Department of Geriatrics Beijing Tsinghua Changgung Hospital Affiliated to Tsinghua University School of Clinical Medicine Tsinghua University Beijing 102218 China; 2Center for Cardiac Intensive Care Beijing Anzhen Hospital Capital Medical University Beijing 100029 China

关键词：三尖瓣下移畸形；右心室功能障碍；心律失常

英文关键词：Ebstein′sanomaly；Rightventriculardysfunction；Arrhythmia

• 摘要：

• 三尖瓣下移畸形（EA）又称Ebstein畸形，是一种罕见且复杂的先天性心脏病。EA由右心室心肌病和三尖瓣病变组成，从而导致患儿右心室功能障碍和心律失常的发病率升高。目前先天性心脏病方面的专家对EA的理解和认知有所提高。但疾病异质性和复杂的治疗方案仍然是巨大挑战。本文对EA的认识与治疗最新进展进行综述。

• Ebstein′s anomaly (EA) is a complex and rare congenital heart disease.  EA is composed of right ventricular cardiomyopathy and tricuspid valve diseases, and increase incidences of right ventricular dysfunction and arrhythmia in children. Currently congenital cardiologists have improved their understanding and awareness of EA. However, heterogeneity and treatment options are still great challenges. This review describes the latest progress in the recognition and treatment of EA.