255例完全性肺静脉异位引流手术治疗的单中心回顾性研究

目的:分析单中心完全性肺静脉异位引流(TAPVC)手术治疗的早中期效果。方法:连续入组2009年1月至2019年10月首都医科大学附属北京儿童医院心脏外科收治并进行手术治疗的0~3岁TAPVC患儿共255例。按照年龄将患儿分为两组:0~6个月组(A组,n=195)和6个月以上~3岁组(B组,n=60),总结其临床特点,并观察两组患儿术后早期死亡(住院期间死亡或放弃治疗或出院后1月内死亡)、中期死亡(出院后且在术后1个月以上死亡)情况以及肺静脉梗阻(PVO)发生情况。结果:手术时患儿的中位年龄为3.9个月(4 h~3岁),中位体重为5.2 kg(2.5~15.0 kg);解剖分型包括:心上型120例(47.1%),心内型110例(43.1%),心下型4例(1.6%),混合型21例(8.2%)。所有患儿均有房间隔缺损或卵圆孔未闭,术前伴PVO 60例(23.5%),重度肺动脉高压(PH)144例(56.5%),中-重度三尖瓣反流152例(59.6%)。术后早期死亡16例(6.3%),均为A组患儿,主要死亡原因为低心排综合征、PH危象导致心力衰竭。239例患儿存活出院,其中有随访资料者237例(99.2%,237/239),随访中位时间为3.6年(1.0个月~11.0年)。中期死亡5例(2.1%,5/237),其中A组4例,B组1例,且均死于术后6个月内,死亡原因主要为术后PVO、重症肺炎、呼吸循环衰竭。术后PVO 31例(13.1%,31/237),其中A组28例(90.3%,28/31),5例(16.1%,5/31)在术后中期死亡。结论:不同年龄段的TAPVC患儿手术治疗效果有明显差异:6个月以上患儿可获得满意的手术治疗效果;≤6个月患儿合并PVO、中重度三尖瓣反流、重度PH的比例均较高,易发生代谢性酸中毒,往往需要急诊手术治疗,手术死亡率较高。PVO是TAPVC矫正术后的严重并发症,也是影响术后中远期生存率的重要因素,低龄患儿术后PVO发生率尤其高。

Efficacy and Outcome of Surgical Treatment of 255 Cases of Total Anomalous Pulmonary Venous Connection:Single Center Experience

Objectives:This study aims to analyze the early and mid-term effects of surgical treatment of total anomalous pulmonary venous connection(TAPVC).Methods:,A total of 255 children with TAPVC aged 0-3 years,who received surgical treatment from January 2009 to October 2019 in our center,were enrolled in this analysis.Patients were divided into two groups according to age,0-≤6 months group(group A,n=195),>6 months-≤3 years group(group B,n=60).The clinical characteristics were analyzed,and incidence of the early postoperative death(death during hospitalization or giving up treatment or death within 1 month after operation),mid-term death(death after discharge and death more than 1 month after operation)and pulmonary venous obstruction(PVO)were observed.Results:The median age during surgery was 3.9 months(4 hours-3 years).The median surgical weight was 5.2 kg(2.5-15.0 kg).Anatomical classification was as follows:120 cases of supracardiac type(47.1%),110 cases of intracardiac type(43.1%),4 cases of subcardiac type(1.6%),and 21 cases of mixed type(8.2%).All children had atrial septal defect or patent foramen ovale,60 cases(23.5%)were accompanied by PVO,and 144 cases(56.5%)with severe pulmonary hypertension,152 cases(59.6%)had moderate to severe tricuspid regurgitation.16 patients(6.3%)died early after operation,all of them were in group A.239 cases survived the operation and discharged,the follow-up rate was 99.2%(237/239),and the median follow-up period was 3.6 years(1.0 month-11.0 years).There were 5 mid-term deaths(2.1%,5/237),4 in group A and 1 in group B,and all died within 6 months after surgery.The main causes of death were postoperative PVO and severe pneumonia,respiratory and circulatory failure.Postoperative PVO was found in 31 cases(13.1%,31/237),28 cases(90.3%,28/31)in group A,and 5 cases(16.1%,5/31)with postoperative PVO died during postoperative follow-up.Conclusions:There are significant differences in post-surgical treatment outcome of TAPVC children with different ages.Post-surgery Outcome is satisfactory in children aged>6 months.Children with TAPVC at age≤6 months are often complicated with PVO,moderate to severe tricuspid regurgitation,severe pulmonary hypertension and prone to metabolic acidosis,which might require emergent surgery and are related high operation mortality.PVO is a serious complication after TAPVC correction,the incidence of PVO is high in young children than children>6 months,and PVO serves as an important factor affecting the long-term survival after surgery.