三尖瓣下移畸形右心室评估与术式选择  

The important index for clinical evaluation of the right ventricle of ebstein's anomaly

作　　者：李晓锋[1] 苏俊武[1] 冷昭廷[1] 刘爱军[1] 范祥明[1] 李志强[1] 贺彦[1] 张晶[1] 刘迎龙[1] 

机构地区：[1]首都医科大学附属北京安贞医院小儿心脏中心,北京市100029

出　　处：《中华全科医学》2014年第9期1367-1369,共3页Chinese Journal of General Practice

基　　金：北京安贞医院院长基金(2012Z02);2011年北京市科技计划项目(Z111100074911001)

摘　　要：目的回顾性分析46例三尖瓣下移畸形的外科治疗结果及术前超声资料,以期为术前评估三尖瓣下移畸形不同手术方式的选择指征提供线索。方法 2010年3月—2013年9月收治三尖瓣下移畸形46例,其中男性28例,女性18例,年龄0.4-35岁,平均体重（26.2±18.6）kg。Carpentier分型A型4例,B型26例,C型15例,D型1例。行双心室矫治33例,一个半心室矫治10例,单纯行双向Glenn术1例,肺动脉环缩术2例。分析全组术前超声资料,对比双心室矫治及一个半心室矫治病例房化右心室与功能右心室参数及手术结果。结果本组术后死亡1例（2.2%）。全组功能右心室/房化右心室比值（FRV/ARV比值）为0.79±0.31,双心室矫治组FRV/ARV比值为0.99±0.35,一个半心室矫治组FRV/ARV比值为0.43±0.23,2组差异存在统计学意义（t=5.9520,P=0.0000）。12例术后出现心包积液（26.09%）,其中双心室矫治组出现5例,一个半心室矫治组7例。34例术后无心包积液患者FRV/ARV为1.07±0.92,术后心包积液患者FRV/ARV为0.49±0.41,2组差异存在统计学意义（t’=2.8897,P〈0.05）。随访2-44个月无死亡,无严重并发症发生。结论三尖瓣下移畸形的手术方式的选择应参考左右心室发育情况,功能右心室/房化右心室比值对抉择三尖瓣下移畸形手术方式可能有一定意义。Objective To explore the indexes for the surgical choice for the treatment of Ebstein＇s anomaly in different pathological classification by analyzing 46 patients. Methods 46 patients with Ebstein＇s anomaly underwent operation from March 2010 to Septermber 2013 were enrolled,with male 28 and female 18. The pathologic classifications from A to D were 4,26,15 and 1 respectively. The preoperative echocardiography data and the operative findings were combined to analyze the surgical strategy decision. Results Excellent surgical results acquired with one early death of arrhythmia.Surgical strategy included two-ventricle repair in 33 patients,One-and-a-Half-ventricle repair in 10 patients,Glenn procedure in 1 patient,and Pulmonary artery Banding in 2 patients. The ratio of functional right ventricle /artrialized right ventricle（ FRV /ARV） in two-ventricle repair group was significant higher than that of One-and-a-Half-ventricle repair group（ t =5. 9520,P =0. 0000）. Conclusion Surgical strategies should be chosen according to the situations of functional RV,FRV /ARV,left ventricle and the tricuspid valve. FRV /ARV may be a good index to evaluate the functional right ventricle of Ebstein＇s anomaly.

关 键 词：先天性心脏病 三尖瓣下移畸形 功能右心室/房化右心室比值 外科治疗 

分 类 号：R541.1[医药卫生—心血管疾病] R725.4[医药卫生—内科学]