2021 年第 4 期 第 16 卷

特发性肺动脉高压患儿全身麻醉下右心导管术中及术后肺动脉高压危象的特点分析

Characteristics of pulmonary hypertensive crisis during and after right cardiac catheterization under general anesthesia in children with idiopathic pulmonary arterial hypertension

作者：王嵘1李强强2张陈2崔博群1马骏1顾虹2

英文作者：Wang Rong1 Li Qiangqiang2 Zhang Chen2 Cui Boqun1 Ma Jun1 Gu Hong2

单位：1首都医科大学附属北京安贞医院麻醉中心100029；2首都医科大学附属北京安贞医院小儿心脏中心100029

英文单位：1Anaesthesiology Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China; 2Pediatric Cardiac Center Beijing Anzhen Hospital Capital Medical University Beijing 100029 China

关键词：肺动脉高压；儿童；右心脏导管术；全身麻醉

英文关键词：Pulmonaryarterialhypertension;Children;Rightcardiaccatheterization;Generalanesthesia

• 摘要：

• 目的 探讨特发性肺动脉高压（IPAH）患儿全身麻醉下右心导管术中及术后发生不良事件风险及肺动脉高压危象（PHC）的特点。方法 收集2008年2月至2019年12月于首都医科大学附属北京安贞医院接受全身麻醉下右心导管术的67例IPAH患儿（其中6例接受2次右心导管术，共73例次）的临床资料进行回顾性分析。根据术中及术后是否发生PHC，分为PHC组（15例，15例次）和非PHC组（52例，58例次）。比较2组的一般资料、术前超声心动图指标、术中血流动力学指标、手术相关情况。采用Kaplan-Meier生存曲线比较PHC患儿与非PHC患儿随访期间生存率。结果 PHC组PAH相关基因突变、晕厥史比例均高于非PHC组［91.7%（11/12）比48.5%（16/33）、53.3%（8/15）比22.4%（13/58）］；PHC组术前右心室内径/左心室内径比值、三尖瓣反流峰值流速均高于非PHC组［（1.08±0.38）比（0.70±0.28）、（4.6±0.7）m/s比（4.1±0.8）m/s］；差异均有统计学意义（均P<0.05）。PHC组术中肺动脉收缩压、肺动脉平均压、右心房压力、肺血管阻力指数均高于非PHC组，心脏指数、急性血管反应试验阳性比例均低于非PHC组，差异均有统计学意义（均P<0.05）。在PHC组中，PHC发生于麻醉诱导期间4例次、右心导管操作期间8例次、肺动脉造影后2例次、术后病房治疗期间1例次，4例次予心脏按压，1例患儿术后76 h死亡。Kaplan-Meier生存曲线分析显示，截至随访36个月，PHC患儿生存率低于非PHC患儿，差异有统计学意义（Log-rank χ2=12.955，P<0.001）。结论 IPAH患儿基础病情重，全身麻醉下右心导管术PHC发生风险高，PHC发作时血流动力学不稳定、进展快，需及时辨别PHC并积极治疗。

• Objective To investigate the characteristics of pulmonary hypertensive crisis (PHC) during and after right cardiac catheterization under general anesthesia in children with idiopathic pulmonary arterial hypertension (IPAH). Methods From February 2008 to December 2019, clinical data of 67 children with IPAH who underwent right cardiac catheterization under general anesthesia （6 children underwent twice right cardiac catheteri-zation, totally 73 times） in Beijing Anzhen Hospital, Capital Medical University were retrospectively analyzed. According to the occurrence of PHC in operation and postoperative period, they were divided into PHC group (15 cases, 15 times) and non-PHC group (52 cases, 58 times). The general data, preoperative echocardiographic indicators, intraoperative hemodynamic parameters, operation related conditions were compared between the two groups. Kaplan-Meier survival curve was used to compare the survival rate during follow-up period between PHC and non-PHC children. Results The proportions of related gene mutation with PAH and history of syncope in PHC group were higher than those in non-PHC group［91.7%（11/12） vs 48.5%（16/33）, 53.3%（8/15） vs 22.4%（13/58）］; value of right ventricular diameter/left ventricular diameter and peak tricuspid regurgitation flow velocity in PHC group were higher than those in non-PHC group［（1.08±0.38） vs （0.70±0.28）,（4.6±0.7）m/s vs （4.1±0.8）m/s］(all P<0.05). Intraoperative pulmonary artery systolic pressure, mean pulmonary artery pressure, right atrial pressure and pulmonary vascular resistance index in PHC group were higher than those in non-PHC group, and cardiac index and positive rate of acute vasodilator test in PHC group were lower than those in non-PHC group(all P<0.05). In PHC group, PHC occurred in 4 times during anesthesia induction, 8 times during right cardiac catheterization, 2 times after pulmonaty angiography and 1 time during postoperative treatment in ward. Four times of cardiac compression were given. A child died 76 h after operation. Kaplan-Meier survival curve analysis showed that, by 36 months of follow-up, the survival rate of children with PHC was lower than that of children without PHC（Log-rank χ2=12.955, P<0.001）. Conclusion Children with IPAH have severe underlying conditions, high risk of PHC during right cardiac catheterization under general anesthesia, unstable hemodynamics at the onset of PHC, rapid progression, and PHC needs to be indentified in time and treated actively.