新生儿期肺动脉闭锁合并室间隔缺损外科治疗的效果评价

Outcome assessment of different surgeries for neonates with pulmonary atresia and ventricular septal defect

目的评价肺动脉闭锁合并室间隔缺损(pulmonary atresia with ventricular septal defect,PA/VSD)新生儿期外科手术的治疗效果,探索PA/VSD患者在新生儿期行外科手术的可行性及手术方式的选择。方法纳入2004年7月至2014年10月在我中心行首次外科手术的14例PA/VSD新生儿。提取其基本特征、术前、手术和术后临床资料及截至2016年10月10日的最近一次随访信息。比较不同手术方式的短、中期生存率和姑息术后的根治率。结果研究对象中,行一期根治术、右室流出道(RVOT)重建姑息术和体肺分流姑息术者分别为4例(28.6%)、6例(42.9%)和4例(28.6%)。首次术后总住院死亡率28.6%(4/14)。中位随访46个月,无死亡病例,5年生存率为71.4%(10/14),纳入患儿的总根治率为64.3%(9/14)。虽然首次手术行一期根治术、RVOT重建术和体肺分流术的死亡率差异无统计学意义(50.0%vs. 33.0%vs. 0.0%,P=0.280),术后生存曲线和风险曲线提示新生儿期首次行体肺分流术者效果更好。首次手术行RVOT重建和体肺分流术的根治率差异无统计学意义(75.0%vs. 50.0%,P=0.470),两者根治距首次姑息术的时间差异无统计学意义(32.0个月vs. 18.0个月,P=0.400)。结论 PA/VSD患儿在新生儿期行外科手术仍是较大的挑战,但只要术后早期生存,此类患者中期生存效果较好。对于不得不在新生儿期外科手术挽救生命的病例,首次手术选择体肺分流姑息术,后期根据患者肺动脉发育情况选择根治术生存效果可能更好。

Objective To explore the feasibility and option of different surgeries for neonates with pulmonary atresia and ventricular septal defect(PA/VSD)through assessing the effect of common surgeries.MethodsFourteen neonates who underwent their first surgery in our center from July2004to October2014were included.Their basic characteristics,operation and pre-and postoperative clinical information were extracted.Follow up was conducted and the last visit was on October10,2016.Short-and midterm survival and total correction rate were compared among different surgeries.ResultsAmong the14patients,there were4(28.6%)patients,6(42.9%)and4(28.6%)who underwent one-stage repair,right ventricular outflow tract(RVOT)reconstruction,and systemic to PA shunt operation respectively.The overall in-hospital mortality after the first operation was28.6%(4/14).At last visit,no death occurred resulting the5-year survival rate of71.4%(10/14).The overall total correction rate for all neonates was64.3% (9/14).Although no statistical difference was found in the mortality among the one-stage repair,RVOT reconstruction and systemic to PA shunt group(50.0%vs.33.3%vs.0.0%,P=0.280),the survival and hazard analysis implied better outcomes of the systemic to PA shunt palliation operation.There was no statistical difference in the total correction rate and months from the first palliative operation to correction between those who underwent RVOT reconstruction and systemic to PA shunt(75.0%vs.50.0%,P=0.470;32.0months vs.18.0months,P=0.400).ConclusionPerforming surgeries for neonates with PA/VSD is still a great challenge.However,the midterm survival rate was optimistic for the early survivors.Systematic to PA shunt seemed to be a better choice with lower mortality for the neonates with PA/VSD Who need the surgery to survive.