婴儿完全性肺静脉异位引流的个性化方案治疗

Individualized Surgical Treatment of Total Anomalous Pulmonary Venous Connection in Infants

目的总结应用个性化手术方案治疗婴儿完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的临床经验。方法2017年1月—2020年12月苏州大学附属儿童医院连续收治的TAPVC并实施手术的婴儿34例。男23例,女11例。年龄1 d~13个月(中位值15 d),其中新生儿21例。体重2.4~7.0 kg(中位值3.6 kg)。分型:心上型10例,心内型13例,心下型6例,混合型5例。术前肺静脉梗阻12例(35%)。急诊手术5例,亚急诊手术7例。所有患儿针对不同解剖类型采用个性化方案实施手术。结果无手术或住院死亡。体外循环时间58~245 min(中位值119 min),16例深低温停循环时间3~50 min(中位值38 min)。呼吸机辅助时间5~252 h(中位值80 h),监护室滞留时间2~45 d(中位值8 d),住院12~54 d(中位值18 d)。术后延迟关胸2例;心包填塞需引流1例;右侧少量气胸1例,自行吸收;腹膜透析2例;一过性快速心律失常5例;Ⅲ度房室传导阻滞植入永久起搏器1例;术后早期出现神经系统一过性症状3例。所有患者均得到随访,随访期6~44个月(中位数25个月)。随访期无死亡,1例心下型患儿术后1个月出现肺静脉梗阻,再次手术恢复良好。其余患儿心功能均为NYHAⅠ级,生长发育良好。结论通过个性化手术方案,婴儿TAPVC可以取得满意的近、中期疗效。

Objective To summarize the clinical experience in the treatment of total anomalous pulmonary venous connection(TAPVC)with individualized surgical method.Methods 34 patients with TAPVC underwent surgical correction from January 2017 to December 2020 in Children's Hospital of Soochow University.Among the 34 patients,23 were males and 11 were females.There were 21 newborns.Ages ranged from 1 day to 13 months,with a median age of 15 days.The weight range is 2.4 to 7.0 kg,with a median weight of 3.6 kg.10 patients had supracardiac connection,13 patients had intracardiac connection,6 patients had infracardiac connection and 5 patients had mixed connection.12(35%)patients presented with pulmonary venous obstruction,and 5 underwent emergent TAPVC repair.All patients were operated on a personalized protocol for different anatomical types.Results No patient died postoperatively.The CPB time ranged from 58 to 245 minutes(median,119 minutes).Deep hypothermic circulatory arrest was used in 16 patients and median time 38 minutes(range,3-50 minutes).Median length of ventilator support was 80 hours(range,5-252 hours).The median cardiac care unit and hospital stay were 8 days(range,2-45 days)and 18 days(range,12-54 days),respectively.The postoperative complications included delayed sternal closure in 2 patients.1 case of cardiac tamponade,and 1 case of right pneumothorax.1 patient had absorbed a small amount of right pneumothorax on her own,the renal function of 2 patients with peritoneal dialysis.5 cases of transient tachyarrhythmia.Third degree atrioventricular block was implanted with a permanent pacemaker in 1 case.There were 3 cases of transient neurological injury in the early postoperative period.All patients were followed up for 6-44 months(median 25 months).No patient died during the follow-up period.1 child presented pulmonary vein obstruction again one month after the operation,reoperation with sutureless technique.All the other children had NYHAⅠgrade of cardiac function and had good growth and development.Conclusion With the individualized surgical scheme,TAPVC can achieve satisfactory short-term and medium-term efficacy in infants.