Ebstein的异常,1866年由威廉·埃布斯坦博士首次描述,占先天性心脏缺陷的0.3-0.5%,占先天性三尖瓣异常的40%。Ebstein异常影响三尖瓣的发育与广泛变化的形态和,因此,临床表现。相关的先天性心脏病变倾向于在年轻患者中更常见,甚至可能是出现的原因。介绍可能与胎儿生命中最极端的形式不同,在成年后期无症状诊断。最有症状的患者在新生儿期需要重症监护支持。本文总结和分析了有关Ebstein异常的文献,并为调查提供了一个框架,管理,以及对这些患者的随访,无论是通过胎儿检测还是在成年后期出现。对于每个年龄组,临床表现,需要诊断性调查,自然史,和管理进行了描述。对Ebstein异常患者可用的手术选择进行了详细的分析,从最初的单叶修复开始到最近的圆锥修复及其修改。该审查还评估了怀孕对Ebstein血液循环的影响,反之亦然,Ebstein对妊娠结局的影响。最后,所附附录中提供了结构化超声心动图方案和对多学科团队全面讨论有用的关键信息.
Ebstein\'s anomaly, first described in 1866 by Dr William Ebstein, accounts for 0.3-0.5% of congenital heart defects and represents 40% of congenital tricuspid valve abnormalities. Ebstein\'s anomaly affects the development of the tricuspid valve with widely varying morphology and, therefore, clinical presentation. Associated congenital cardiac lesions tend to be found more often in younger patients and may even be the reason for presentation. Presentation can vary from the most extreme form in fetal life, to asymptomatic diagnosis late in adult life. The most symptomatic patients need intensive care support in the neonatal period. This article summarizes and analyzes the literature on Ebstein\'s anomaly and provides a framework for the investigation, management, and follow-up of these patients, whether they present via fetal detection or late in adult life. For each age group, the clinical presentation, required diagnostic investigations, natural history, and management are described. The surgical options available for patients with Ebstein\'s anomaly are detailed and analyzed, starting from the initial mono-leaflet repairs to the most recent cone repair and its modifications. The
review also assesses the effects of pregnancy on the Ebstein\'s circulation, and vice versa, the effects of Ebstein\'s on pregnancy outcomes. Finally, two attached appendices are provided for a structured echocardiogram protocol and key information useful for comprehensive Multi-Disciplinary Team discussion.