Holst Kimberly A, Connolly Heidi M, Dearani Joseph A
MAYO CLINIC, ROCHESTER, MINNESOTA.
Methodist Debakey Cardiovasc J. 2019 Apr-Jun;15(2):138-144. doi: 10.14797/mdcj-15-2-138.
Ebstein's anomaly is a malformation of the tricuspid valve with myopathy of the right ventricle (RV) that presents with variable anatomic and pathophysiologic characteristics, leading to equally variable clinical scenarios. Medical management and observation is often recommended for asymptomatic patients and may be successful for many years. Tricuspid valve repair is the goal of operative intervention; repair also typically includes RV plication, right atrial reduction, and atrial septal closure or subtotal closure. Postoperative functional assessments generally demonstrate an improvement or relative stability related to degree of RV enlargement, RV dysfunction, RV fractional area change, and tricuspid valve regurgitation.
埃布斯坦畸形是一种三尖瓣畸形合并右心室心肌病，具有可变的解剖和病理生理特征，导致同样多变的临床情况。对于无症状患者，通常建议进行药物治疗和观察，并且这种治疗可能在多年内有效。三尖瓣修复是手术干预的目标；修复通常还包括右心室折叠、右心房缩小以及房间隔闭合或部分闭合。术后功能评估通常显示与右心室扩大程度、右心室功能障碍、右心室面积变化分数以及三尖瓣反流相关的改善或相对稳定。
