新生儿室间隔完整型肺动脉闭锁治疗结果  

Treatment outcomes of pulmonary atresia with intact ventricular septum in newborns

作　　者：陶曙光[1] 姚俊平[1] 谷疆蓉[1] 杨仕海[1] 温林林[1] 金立臣[1] 高伟 TAO Shuguang;YAO Junping;GU Jiangrong(Hebei Children’s Hospital,Hebei,Shijiazhuang 050031,China)

机构地区：[1]河北省儿童医院,石家庄市050031

出　　处：《河北医药》2024年第16期2436-2441,共6页Hebei Medical Journal

摘　　要：目的 总结室间隔完整型肺动脉闭锁患儿的治疗经验。方法 回顾性分析2017年1月至2023年1月收治的16例新生儿室间隔完整型肺动脉闭锁临床资料,男9例,女7例;日龄4 h~25 d;入院体重1.6~4 kg,平均(3.22±0.65) kg。所有患儿初期治疗后进行随访,随访时间3个月至6年,中位随访时间33(12,55)月。初期治疗措施包括以下术式:体外循环下房间隔扩大、B-T分流、镶嵌治疗、非体外下经胸肺动脉瓣切开术等,每例患儿选择一种或者多种组合术式。结果 患儿院内死亡1例,院外无死亡病例。再次治疗6例,其中行经导管肺动脉瓣球囊扩张成形术5例,行双向Glenn术1例。其余9例患儿术中后已5年1例,经皮血氧饱和度>95%,房间隔转为左向右分流,远期免于再干预,其余患儿仍在随访中,存在再次干预可能。首次根治后1个月较大的肺动脉根部直径远期行双心室根治可能性大。结论 室间隔完整型肺动脉闭锁是需要序贯性治疗和观察的疾病,为获得较好的生存质量,初次治疗应依据右心室发育程度选择个性化措施,然后密切主动随访,适时给予再次治疗。Objective To summarize the treatment experiences in pulmonary atresia with intact ventricular septum(PA/IVS)in newborns.Methods Clinical data of 16 newborns with PA/IVS treated between January 2017 and January 2023 were retrospectively analyzed.There were 9 males and 7 females,with the age ranging from 4 hours to 25 days,body weight on admission ranging from 1.6kg to 4.0kg and a mean weight of(3.22±0.65)kg.All participants were followed up for a median of 33(12,55)months,ranging from 3 months to 6 years.Treatment approaches included atrial septum enlargement surgery under an extracorporeal circulation,BT shunt,hybrid therapy,and transthoracic pulmonary valve incision without cardiopulmonary bypass.One or more surgeries was performed in each neonate.Results One of the 16 children died in the hospital,and there were no deaths outside the hospital.Six cases were re-treated,including 5 treated with balloon valvuloplasty and 1 with bidirectional Glenn procedure.Among the remaining 9 newborns,1 has been lived 5 years post-operatively without the need of re-interventions,and the transcutaneous oxygen saturation was 95%and above and the interatrial septum returned a left-to-right shunt.The remaining newborns have been followed up,and they may have re-interventions.After one month of the first radical treatment,there was a large possibility to perform a biventricular radical surgery later for a larger diameter of the pulmonary artery root.Conclusion Sequential therapy and observation for PA/IVS are necessary.An individualized initial intervention based on the development of the right ventricle plus a regular active follow-up for a potential re-intervention ensure a high quality of life.

关 键 词：室间隔完整型肺动脉闭锁 新生儿 镶嵌治疗 外科治疗 随访 

分 类 号：R725.411[医药卫生—儿科]