特发性肺动脉高压患儿心导管检查及急性肺血管反应试验效果评价

Cardiac catheterization and pulmonary vasoreactivity testing in children with idiopathic pulmonary arterial hypertension

目的 探讨对特发性肺动脉高压（IPAH）患儿进行心导管检查及急性肺血管反应试验的临床价值、药物选择及结果评价.方法 2009年4月至2013年9月收住北京安贞医院小儿心脏中心拟诊IPAH、年龄18岁以下、世界卫生组织（WHO）心功能Ⅱ至Ⅲ级的患儿,分别行左、右心导管检查及吸入纯氧和伊洛前列素（PGI2）的急性肺血管反应试验,分析血流动力学指标变化,并分别应用两种阳性标准对试验结果进行评价.结果 39例拟诊IPAH的患儿接受了心导管检查,4例排除IPAH,4例术中发生肺动脉高压危象,另31例进行了规范的心导管检查和肺血管反应试验.基础肺动脉平均压（mPAP） （66±16） mmHg（1 mmHg=0.133 kPa）,肺血管阻力指数（PVRI）（17±8）WoodU·m^2;吸入纯氧后mPAP降至（59±16） mmHg,PVRI降至（14±8）Wood U·m^2;吸入PGI2后mPAP降至（49±21）mmHg,PVRI降至（12±9）Wood U· m^2,与基础水平比较,差异均有统计学意义（f=4.88、4.56,7.04、6.33,均P＜0.001）.根据Sitbon标准,吸氧试验阳性率6.5％ （2/31）,PGI2吸入试验阳性率35.5％ （11/31）,差异有统计学意义（Х^2 =7.11,P=0.004）.根据Barst标准,吸氧试验阳性率16.1％ （5/31）,PGI2吸入试验阳性率51.6％（16/31）,差异有统计学意义（Х^2 =9.09,P=0.001）.结论 心导管检查及急性肺血管反应试验对于IPAH患儿鉴别诊断、病情判断、治疗方案包括抢救方案的选择均具有重要价值.肺动脉高压危象是IPAH患儿心导管检查的重要并发症.PGI2可作为较理想的肺血管反应试验药物应用于IPAH患儿,其阳性率明显大于传统的纯氧;不同阳性标准的肺血管反应试验结果不完全一致,临床上应根据治疗目的进行综合评价.

Objective As an important method of hemodynamic assessment in idiopathic pulmonary arterial hypertension （IPAH）,cardiac catheterization combined with pulmonary vasoreactivity testing remains with limited experience in children,and the acute pulmonary vasodilator agents as well as response criteria for vasoreactivity testing remain controversial.The aim of this study was to investigate the clinical importance,agent selection,and responder definition of cardiac catheterization combined with pulmonary vasoreactivity testing in pediatric IPAH.Method The patients admitted to Department of Pediatric Cardiology of Beijing Anzhen Hospital between April 2009 and September 2013 with suspected IPAH,under 18 years of age,with WHO functional class Ⅱ or Ⅲ,were enrolled.All the patients were arranged to receive left and right heart catheterization and pulmonary vasoreactivity testing with inhalation of pure oxygen and iloprost （PGI2） respectively.Hemodynamic changes were analyzed,and two criteria,the European Society of Cardiology recommendation criteria （Sitbon criteria） and traditional application criteria （Barst criteria）,were used to evaluate the test results.Result Thirty-nine cases of children with suspected IPAH underwent cardiac catheterization.In 4 patients IPAH was excluded; 4 patients developed pulmonary hypertension crisis.The other 31 patients received standard cardiac catheterization and pulmonary vasoreactivity testing.Baseline mean pulmonary artery pressure （mPAP） was （66 ± 16） mmHg （1 mmHg =0.133 kPa）,and pulmonary vascular resistance index （PVRI） （17 ± 8） Wood U m^2.After inhalation of pure oxygen,mPAP fell to （59 ± 16） mmHg,and PVRI to （14 ± 8） Wood U · m^2 （t =4.88 and 4.56,both P 〈0.001）.After inhalation of PGI2,mPAP fell to （49 ±21） mmHg,and PVRI to （12±9） Wood U · m^2（t =7.04 and 6.33,both P 〈0.001）.According to the Sitbon criteria,the proportion of pure oxygen responders was 6.5% （3/31）,while PGI2 responders was 35.5%,and the difference was significant （P =0.004）.According to the Barst criteria,the proportion of pure oxygen responders was 16.1% （5/31）,while PGI2 responders was 51.6% （16/31）,and the difference was significant （Х^2 =0.09,P =0.001）.Conclusion For children with IPAH,cardiac catheterization combined with pulmonary vasoreactivity testing has important value in differential diagnosis,severity estimation,and treatment （including the emergency treatment） choices.Pulmonary hypertension crisis is an important complication of cardiac catheterization in pediatric IPAH.Younger age,general anesthesia,crisis history,and poor heart function are important risk factors for pulmonary hypertension crisis.PGI2 is a relatively ideal agent for vasoreactivity testing in children with IPAH,which has more responders than traditionally used pure oxygen.Results of responders are not completely consistent using different criteria,and comprehensive evaluation should be done according to the goals of treatment in clinical practice.