新生儿复杂先天性心脏病15年治疗分析  

Surgical outcomes of neonates with critical and complex congenital heart diseases in a single center:a 15-year experience

作　　者：张惠丽 李守军[1] 闫军[1] 王旭[1] 花中东[1] 王强 Zhang Huili;Li Shoujun;Yan Jun;Wang Xu;Hua Zhongdong;Wang Qiang(Center for Pediarie Cardine Surgery,Furai Hospital,Chinese Academy of Medical Science and Peking Union Medical College,National Center for Cardiorascupr Diseases,Bejjing 100037,China.)

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

出　　处：《中华新生儿科杂志（中英文）》2019年第6期401-407,共7页Chinese Journal of Neonatology

基　　金：十三五国家重点研发计划(2017Y FC1308100)。

摘　　要：目的总结近15年新生儿复杂先天性心脏病(简称先心病)的治疗经验,探讨新生儿复杂先心病的手术时机和预后。方法回顾性分析2004年1月至2018年12月中国医学科学院阜外医院小儿外科中心诊断并在新生儿期行手术治疗的复杂先心病患儿资料。根据治疗时间分为2004—2008年组、2009—2013年组和2014—2018年组。所有病例均经超声心动图诊断,部分患儿同时行多排螺旋C T检査。随访结果依据门诊复查和电话随访。结果共纳人357例,其中男261例,女96例;手术时体重2100〜4900 g;手术时日龄2〜28 d,其中在7 d手术16.0%(57/357);胎儿期诊断13.4%(48/357)。生后表现为紫绀315例(88.2%),其中经皮血氧饱和度<0.6的患儿占19.0%(60/315);杂音181例(50.7%)。完全性大动脉转位221例(61.9%),其中室间隔完整型147例;完全性肺静脉异位引流55例(15.4%);肺循环依赖动脉导管开放的先心病43例(12.0%);体循环依赖动脉导管开放的先心病20例(5.6%);其他18例(5.0%)。手术一期矫治299例(83.8%),姑息手术58例(16.2%)。医院内死亡28例(7.8%)。从前到后3个时间段胎儿期诊断率逐步上升,分别为3.0%(2/67)、6.3%(7/111)、21.8%(39/179);手术后医院内死亡率,尤其完全性大动脉转位的医院内死亡率逐步降低,分别为11.9%(8/67)、5.4%(6/111)、2.8%(5/179)。存活出院329例,7例失访,随访率97.9%(322/329),随访期限63个月(3〜183个月)。随访期间死亡5例。9例行二次矫治手术,3例因左心室或右心室流出道狭窄再次手术。存活患儿生长发育良好,心功能均为I级。结论新生儿复杂先心病多表现为紫绀,近15年来胎儿期诊断率逐渐增加,手术后医院内死亡率,尤其完全性大动脉转位的医院内死亡率逐渐降低。绝大部分危重复杂先心病患儿可以在新生儿期行一期矫治手术,手术成功率高、手术治疗效果好。姑息手术在分期治疗复杂先心病中仍起重要作用。Objective To summarize the surgical outcomes of neonates with critical and complex congenital heart diseases in the last 15 years to determine the optimal operation time and improve the prognosis.Method From January 2004 to December 2018,data of patients with complex congenital heart disease(CHD)who received surgery in our center were retrospectively analysed.All patients were diagnosed using echocardiography,some patients also received multidetector computed tomography(MDCT)examination.According to the operation date,patients were assigned into three groups:2004-2008,2009-2013 and 2014-2018 groups.Follow-up data were obtained from outpatient check-up and telephone interview.Result A total of 357(261 males and 96 females)neonates were included.They received the operation at 2-28 days after birth and their body weights were 2100~4900 g.13.4%(48/357)were diagnosed before birth.Percutaneous oxygen saturation(SpO2)<0.95 were detected in 315 patients(88.2%,315/357),among whom 60 had SpO2≤0.60(19.0%,60/315).Cardiac murmur were detected in 181 patients(50.7%,181/357).Among the 357 patients,221 neonates had transposition of the great arteries(TGA),55 had total abnormal pulmonary venous connection(TAPVC),43 had pulmonary atresia and intact ventricular septum(PA/IVS)and severe pulmonary stenosis and intact ventricular septum(SPS/IVS),20 had severe coarctation of the aorta(CoA)/interrupted aortic arch(IAA)/severe aortic stenosis(AS)/hypoplastic left heart syndrome(HLHS)and 18 had other types of complex CHD.The rate of onestage repair was 83.8%(299/357)and 58 neonates underwent palliative surgery.28 patients died in hospital(7.8%,28/357).The rate of prenatal diagnosis in three groups increased[3.0%(2/67),6.3%(7/111),21.8%(39/179)]and in-hospital mortality of all patients,especially patients with TGA,were remarkably decreased in the last 15 years[11.9%(8/67),5.4%(6/111),2.8%(5/179)].329 patients survived after surgery and discharged.The follow-up rate was 97.9%(322/329),7 patients were lost during follow-up,the median durat

关 键 词：心血管畸形 先天性心脏病 手术治疗 婴儿 新生 

分 类 号：R72[医药卫生—儿科]