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目的 调查行二期全腔静脉肺动脉连接术(Fontan术)患儿各手术阶段的生长发育情况.方法 回顾性分析2012年1月至2015年12月我院行二期Fontan术152例患儿的临床资料,其中男84例、女68例,年龄4～16岁.评估患儿各手术阶段的身高、体重,并转换为WHO Z评分,描述患儿的生长发育模式.结果 患儿各手术阶段年龄别体重Z评分(weight for age Z-score,WAZ)、年龄别身高Z评分(height for age Z-score,HAZ)出生时分别为-0.73和0.06,双向腔肺吻合术(Glenn术)前分别为-1.27±1.37和-1.27±1.72,Fontan术前分别为-0.47±1.08和-0.69±1.17,术后随访时分别为-0.42±1.18和-0.39±1.48.其中Glenn术前与出生时、二期Fontan术前的WAZ、HAZ差异有统计学意义(P＜0.05),Fontan术前与随访时HAZ评分差异有统计学意义(P＜0.05).结论 二期Fontan术患儿经过各手术阶段后其长发育模式发生变化.在Glenn术前出现生长低谷,术后患儿开始迅速追赶生长;至Fontan术前患儿身高、体重尚未达到正常水平,术后患儿追赶生长减慢;并且随访时仍然存在低体重和生长迟缓现象.未来需要采取有效措施如加强Glenn术前患儿的营养支持,以期待改善患儿的生长发育.“,”Objective To investigate somatic growth of children undergoing stage Ⅱ Fontan procedure.Methods Clinical data of 152 children who underwent stage Ⅱ Fontan procedure in Shanghai Children's Medical Center,Shanghai Jiaotong University School of Medicine between January 2012 and December 2015 were reviewed.There were 84 males and 68 females at age of 4-16 years.The weight and height of those children in each operative stage were assessed,and WHO Z-score was obtained to describe the pattern of growth of the children.Results Mean weight for age Z-score (WAZ) and height for age Z-score (HAZ) were-0.73 and 0.06 at birth,-1.27±1.37 and -1.27±1.72 before Glenn procedure,-0.47±1.08 and-0.69±1.17 before Fontan procedure,-0.42±1.18 and-0.39±1.48 at follow-up,respectively.A significant decline (P＜0.05) in WAZ and HAZ was found before Glenn procedure.After the Glenn procedure,there was significant catch-up growth (P＜0.05).The WAZ was stabilized after Fontan procedure,while the HAZ was reversed (P＜0.05) continually.Conclusion The somatic growth is impaired in children with stage Ⅱ Fontan procedure.There is a trough of growth before Glenn procedure.Although catch-up growth occurs after Glenn procedure,low body weight and growth retardation remain at follow-up.Effective interventions such as more intensive nutritional strategies before Glenn procedure should be targeted at those children in the future,so as to improve their growth.