经右心室流出道肺动脉瓣球囊成形治疗室间隔完整型肺动脉闭锁的疗效分析  

Efficacy of surgical balloon valvuloplasty via right ventricular outflow tract for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum

作　　者：马凯[1] 祁磊[1] 逄坤静[1] 张本青[1] 芮璐[1] 林野[1] 刘锐[1] 张森[1] 王官玺 冯子聪 李守军[1] MA Kai;QI Lei;PANG Kunjing;ZHANG Benqing;RUI Lu;LIN Ye;LIU Rui;ZHANG Sen;WANG Guanxi;FENG Zicong;LI Shoujun(Pediatric Cardiac Surgery Center,Fuwai Hospital,National Center for Cardiovascular Disease,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing,100037,P.R.China)

机构地区：[1]中国医学科学院,北京协和医学院,国家心血管病中心,阜外医院小儿外科中心,北京100037

出　　处：《中国胸心血管外科临床杂志》2020年第5期498-502,共5页Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

基　　金：国家自然科学基金资助项目(81700290);“十三五”国家重点研发计划资助项目(2017YFC1308100)。

摘　　要：目的分析经右心室流出道肺动脉瓣球囊成形术(surgical balloon valvuloplasty,SBV)进行右心室减压联合个体化分流手术治疗室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的中远期疗效。方法回顾性分析2005年1月至2017年12月在本中心接受SBV治疗的91例PA/IVS患者的临床资料,其中男52例(57.1%)、女39例(42.9%),中位年龄3个月(1 d,24个月),中位体重4.1(2.5,12.0)kg。结果所有患者均行SBV,其中62例患者同期行其他外科手术,包括动脉导管未闭(patent ductus arteriosus,PDA)结扎术33例;PDA结扎术+改良Blalock-Taussig分流术23例;PDA结扎术+双向格林手术6例。没有早期死亡。中位随访时间8.8(2.5,13.4)年,4例(4.4%)失访。随访患者中,7例(8.0%)患者死亡,1例(1.1%)患者因肺动脉瓣狭窄再次行SBV。在随访中,5例(5.7%)行一个半心室矫治术,2例(2.3%)行Fontan手术;平均三尖瓣Z值为?1.7±1.5,较术前明显增加(t=5.587,P<0.001)。结论通过SBV联合个体化分流手术策略治疗PA/IVS是安全、有效的,大多数患儿可达到双心室矫治,避免单心室姑息治疗。Objective To analyze the mid-long-term outcomes of surgical balloon valvuloplasty(SBV)for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum(PA/IVS).Methods Clinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed,including 52(57.1%)males and 39(42.9%)females.The median age was 3 months(1 d,24 months)and the median weight was 4.1(2.5,12.0)kg.Results The SBV was performed in all patients,and 62 of whom received other simultaneous surgeries,including ligation of patent ductus arteriosus(PDA,33 patients),ligation of PDA with modified Blalock-Taussig shunt(23 patients),ligation of PDA with bidirectional Glenn shunt(6 patients).There was no early postoperative death.The median follow-up time was 8.8(2.5,13.4)years,4 patients were lost.There were 7(8.0%)deaths and 1(1.1%)patient with a re-SBV for pulmonary stenosis.The one and a half ventricular repair was performed in 5(5.7%)patients and Fontan procedure in 2(2.3%)patients.In addition,the mean Z-value of tricuspid valve annulus was?1.7±1.5,which was significant bigger than that before the operation(t=5.587,P<0.001).Conclusion SBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable.The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.

关 键 词：室间隔完整型肺动脉闭锁 肺动脉瓣球囊成形术 预后 外科手术 

分 类 号：R726.5[医药卫生—儿科]