儿童先天性心脏病合并重度肺动脉高压根治术随访分析

Follow-up analysis of congenital heart disease complicated with severe pulmonary arterial hypertension in children

目的:通过对儿童非限制性左向右分流型先天性心脏病合并重度肺动脉高压根治术后的随访,分析超手术指征患儿术后的生存现状并探讨手术指征。方法:收集2005年1月至2016年6月,在我院进行手术治疗的左向右分流型先天性心脏病合并重度肺动脉高压的儿童患者临床资料,所有患儿均有经心导管检查获得的血流动力学资料,随访其术后症状、体征、超声心动图。按术前肺血管阻力指数(PVRI)<6WU/m^2和PVRI≥6WU/m^2分为两组,根据患儿生活质量情况进行生存分析并做Logrank检验,再根据术后是否存在肺动脉高压绘制ROC曲线。结果:59例患儿(男性27例,女性32例),平均8.4岁,主要诊断为室间隔缺损、动脉导管未闭、房间隔缺损、完全型心内膜垫缺损、主肺动脉窗、高压型右室双出口等左向右分流型先天性心脏病,随访时间4~127个月平均69.3个月。PVRI<6WU/m^2组12例(男性6例,女性6例),失访3例,所有患儿随访均无术后PAH;PVRI≥6WU/m^2组患儿47例(男性21例,女性26例),失访15例,除4例患儿随访超声心动图显示无肺动脉高压外,均有不同程度术后肺动脉高压。比较两组患儿无阳性事件的生存曲线,PVRI<6WU/m^2组明显优于PVRI≥6WU/m^2组(Log-rank检验P=0.0496),ROC曲线显示PVRI是预判手术后是否存在肺动脉高压的敏感指标(曲线下面积0.90),且PVRI以8.15WU/m^2为界点时,敏感度为84.2%,特异度为80%。结论:PVRI是评价手术远期预后的关键指标;PVRI<6WU/m^2的患儿手术预后明显好于PVRI≥6WU/m^2的患儿。

Abjective：To analyze the survival status of children out of indication and to discuss the indication through follow-up on children with severe pulmonary hypertension associated with unrestrictive left-right shunt congenital heart disease.Methods：Hemodynamic data and other information are collected from the Children with severe pulmonary arterial hypertension associated with congenital heart disease and treated in Anzhen hospital from January 2005 to June 2016.The survival analysis and log-rank test are executed as well as ROC curve.Results：The object of observation is 59 children （aged 8.4 ± 5.2 years）,27 males and 32 females included,with major cardiac malformations of unrestrictive left-right shunt CHD,and follow-up time of 4-127 months mean （69.3 ＆#177; 33.5）months.12 children belong to Group PVRI 〈 6WU/m2 （6 male and 6 female）,3is lost to follow up,and none patient with postoperative PAH.Comparatively,47 patients belong to Group PVRI ≥6WU/m2 （21 male and 26 female）,15 lost to follow up,and most of whom still suffered pulmonary arterial hypertension （one of them died 10 months after surgery） except 4 patients.According to postoperative living quality,the prognosis of Group PVRI 〈 6WU/m2 was significantly better than group PVRI ≥ 6WU/m2 （Log-rank test P =0.0496） with the rate of no positive event 86.6% in 3-year,79.2% in 5-year and 22.5% in 10-year.Moreover,PAH may get worse if surgeries are operated on the children out of indication.ROC curve demonstrates that PVRI is a sensitive indicator to predict post-operative pulmonary arterial hypertension（AUC 0.90）,with the sensitivity of 84.2%,the specificity of 80.0%,as the boundary point of PVRI 8.15 WU/m2.Conclusion：PVRI is a sensitive indicator for evaluating the long-term prognosis of CHD-PAH surgery.L ong-term surgical outcomes is better in Group PVRI 〈 6WU/m2 than in Group PVRI≥6WU/m2.