不同术式治疗Ebstein畸形临床效果分析

Clinical analysis of different surgical methods for Ebstein malformation

目的通过随访研究,评价不同术式对Ebstein畸形外科治疗的临床效果。方法回顾性分析自2008年6月至2017年6月在北部战区总医院进行手术治疗的98例Ebstein畸形患者的随访资料。根据年龄将患者分为小儿组(年龄<12岁,n=27)和成人组(年龄> 12岁,n=71)。全组患者均由同一医师应用PHilips IE33型号机器采集超声心动图数据,观察两组患者采用不同术式治疗后的三尖瓣返流、过三尖瓣血流、右室面积变化率(FAC)及三尖瓣舒张早期血流峰值速度/舒张末期血流峰值速度(E/A)情况。结果小儿组Danielsons、Carpentier术式近期效果较好,中-远期结果不佳; Cone组近期效果较好。成人组各术式近期结果满意;中-远期三尖瓣置换术(TVR)右室功能明显优于Danielsons、Carpentier术式; Cone组近期结果满意,但缺乏中-远期随访结果。结论 Ebstein畸形术后早期各术式疗效相当,中-远期效果差距较大。非解剖修复(单瓣修复)、术后过三尖瓣偏心血流提示非解剖修复的瓣叶重合是导致术后三尖瓣关闭不全、右心功能发育不良的一个重要原因。Cone重建术由于最接近三尖瓣正常解剖形态,血流位于中央处,三尖瓣瓣叶可完全对合,临床效果好。成人TVR手术效果中-远期效果满意,需规律抗凝。

Objective To evaluate the effect of different surgical methods for Ebstein malformation through follow-up study.Methods A retrospective study was performed on 98 cases of patients with Ebstein malformation who were admitted from June 2008 to June 2017.According to the age, the patients were divided into the pediatric group （age less than 12 years old, n =27） and the adult group （age more than 12 years old, n =71）.All the patients were performed by the same doctor using the PHilips IE33 model machine to collect the number of echocardiography.According to the results,the main observation of three tip regurgitation,over three tip blood flow,FAC,E/A index was performed in the two groups after different surgical procedures.Results The short-term effects of Danielsons and Carpentier were good in the children group,and the medium-long-term results were not good.The Cone group did well in the near term.The short-term results of each operation in adult group were satisfactory.Middle-long term tricuspid valve replacement（TVR） was superior to Danielsons and Carpentier.The recent results of Cone group were satisfactory,and the results of medium-long-term follow-up were absent.Conclusion The results are similar in the early stage after Ebstein malformation,and the middle-long term result is large.The non dissection repair（single repair）,and the undissected reunion of the undissected flap after the operation shows the three apex insufficiency,an important cause of the right ventricular dysplasia.Cone reconstruction is close to the normal anatomical morphology of tricuspid valve and blood flow is located in the center.The long-term effect of adult TVR surgery is satisfactory,requiring regular anticoagulation.