Analysis of Surgical Treatment of 20 Cases of Ebstein’ s Anomaly Combined with Atrial Septal Defect
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摘要:
目的 分析三尖瓣下移畸形合并房间隔缺损外科手术治疗处理策略及近期随访结果。 方法 回顾性分析2017年9月至2021年6月诊治的20例三尖瓣下移畸形合并房间隔缺损患者的临床资料及随访结果，并对该组患者术前及术后超声心动图结果进行统计分析。 结果 16例患者选择双心室矫治术，其中采用水平房化心室折叠三尖瓣成形术（Danielsons术式）2例，采用垂直房化心室折叠三尖瓣成形术（Carpentier术式）4例，锥形重建术10例；2例患者选择一个半心室矫正术（三尖瓣成形术加双向Glenn手术）；2例患者行双向Glenn术。20例患者合并的房间隔缺损在进行体外循环矫治畸形时均一期闭合。术后1、3、6、12个月，患者右心室大小较术前明显缩小（P < 0.05），心功能（左心室射血分数）明显提高（P < 0.05）。 结论 三尖瓣下移畸形合并房间隔缺损手术治疗应遵循个体化的治疗原则，双心室矫治术仍是首选的治疗方法，一期关闭房间隔缺损，可以获得良好的治疗效果。 Abstract:Objective To analyze the treatment strategy of the atrial septal defect in the surgical treatment of Ebstein’ s anomaly combined with the atrial septal defect and the short-term follow-up results of the treatment of Ebstein’ s anomaly. Methods A retrospective analysis of the clinical data and follow-up results of 20 patients with Ebstein’ s anomaly and atrial septal defect was conducted from September 2017 to February 2021. And the statistical analysis on the preoperative and postoperative echocardiography results of this group of patients was performed. Results Sixteen patients underwent the biventricular correction surgery, among whom two cases underwent the horizontal atrial tricuspid valvuloplasty（Danielsons procedure）, four cases underwent the vertical atrial tricuspid valvuloplasty（Carpentier procedure）, and ten cases underwent the conical reconstruction. Two patients were given a half ventricular correction surgery（tricuspid valve reconstruction combined with bidirectional Glenn surgery） and two patients underwent the bidirectional Glenn surgery. The combined atrial septal defects were closed in one stage during extracorporeal circulation for correction of deformitie in 20 patients. At 1, 3, 6, and 12 months after the surgery, the patient’ s right ventricular size significantly decreased compared to preoperative（P < 0.05）, and cardiac function（left ventricular ejection fraction） significantly improved（P < 0.05）. Conclusion The surgical treatment of Ebstein’ s anomaly combined with the atrial septal defect should follow the principle of individualized treatment. Biventricular correction is still the first choice for the treatment of Ebstein’ s anomaly, and the atrial septal defect should be closed at one stage, so as to obtain a good therapeutic effect. -
表 1 三尖瓣下移畸形合并房间隔缺损患者术前及术后心脏大小及心功能情况[（$ \bar x \pm s $）/M（Q25，Q75）]
Table 1. Cardiac size and function of patients with Ebstein’s anomaly combined with atrial septal defect before surgery and after surgery[（$ \bar x \pm s $）/M（Q25，Q75）]
时间节点 n 左心房（mm） 左心室（mm） 右心室（mm） 左心室射血分数（%） 术前 20 22.40 ± 4.17 31.70 ± 7.33 32.00（26.25，44.75） 56.00（55.00，60.00） 术后1个月 20 21.50 ± 4.87* 30.75 ± 8.05* 27.00（22.00，39.50）* 60.00（60.00，63.75）* 术后3个月 20 21.55 ± 6.02 31.00 ± 6.31 20.50（16.00，29.00）* 66.00（65.00，68.00）* 术后6个月 20 19.45 ± 4.99 30.20 ± 6.50 19.50（18.00，24.25）* 67.50（65.00，70.00）* 术后12个月 19 19.50 ± 4.48 29.45 ± 6.21 19.00（17.00，22.75）* 67.85（65.24，70.02）* 与术前比较，*P < 0.05。 
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