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目的 探索肺动脉压力对功能性单心室患儿Fontan术后早期转归的影响.方法 回顾性分析2015年1月至2016年12月我院行Fontan术43例患儿的临床资料,其中男24例、女19例,中位年龄4.3(2.5～4.8)岁,术前经心导管测压评估有无肺动脉高压将患儿分为非肺动脉高压组(23例,男16例、女7例)和肺动脉高压组(20例,男8例、女12例).比较两组术后转归的差异.结果 非肺动脉高压组患儿术后无死亡病例,肺动脉高压组患儿死亡率20.0％ (4/20),明显高于非肺动脉高压组患儿(x2=5.34,P=0.02).肺动脉高压组术后中心静脉压(t=-2.50,P=0.02)、N末端前脑钠肽(NT-proBNP)(Z=-3.50,P＜0.01)、腹膜透析率(x2=5.40,P=0.02)、心律失常发生率(x2=4.40,P=0.03)明显高于非肺动脉高压组.肺动脉高压组患儿术后早期肺血管靶向性药物的使用率明显高于非肺动脉高压组(x2=6.30,P=0.04).结论 肺动脉高压对功能性单心室患儿Fontan术后的早期预后有影响.“,”Objective To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation.Methods Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016.There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years.The pulmonary arterial pressure was evaluated by cardiac catheterization.There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females,while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females.Postoperative parameters related to outcomes were compared between the two groups.Results There was no death in the non-PAH group,but the mortality of children in the PAH group was 20.0％ (4/20,x2=5.34,P=0.02).The central venous pressure (t=-2.50,P=0.02),N-terminal prohormone of brain natriuretic peptide (NT-proBNP,Z=-3.50,P＜0.01),peritoneal dialysis rate (x2=5.40,P=0.02),incidence of arrhythmia (x2=4.40,P=0.03) in the PAH group were significantly higher than those of the non-PAH group.The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (x2=6.30,P=0.04).Conclusion Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.