心导管检查对小儿不同类型肺动脉高压的诊治价值分析  

Value of cardiac catheterization in diagnosis and treatment of different types of pediatric pulmonary ar⁃tery hypertension

作　　者：李博宁[1] 刘琮[1] 谢颖[1] 欧振恒 辛颖 李薇玢[1] 姚静[1] LI Bo-ning;LIU Cong;XIE Ying;OU Zhen-heng;XIN Ying;LI Wei-bin;YAO Jing(Department ofCardiology,Shenzhen Children′sHospital,Shenzhen,Guangdong 518038,China)

机构地区：[1]深圳市儿童医院心血管内科,广东深圳518038

出　　处：《岭南心血管病杂志》2021年第4期452-456,共5页South China Journal of Cardiovascular Diseases

基　　金：深圳市三名工程项目(项目编号:SZSM201612057);深圳市战略性新兴产业发展专项基金“创业链+产业链”融合专项扶持技术(项目编号:20180309175312565)。

摘　　要：目的总结不同类型肺动脉高压患者心导管检查结果,分析其特点,为临床诊断及治疗提供参考。方法回顾性分析6例肺动脉高压患者的临床症状,心脏超声检查结果,心导管检查结果[包括肺动脉压力与主动脉压力比值、肺动脉阻力指数(pulmonary vascular resistance index,PVRI)、选择性肺小动脉造影(selective pulmonary arteriography,SPA)、肺循环时间(pulmonary circulation time,PCT)、急性肺血管扩张试验(acute pulmonary vasodilation test,AVT)等]以及治疗效果、随访等资料。结果患者1提示室间隔缺损合并动力型肺动脉高压。患者2为室间隔缺损合并非动力性肺动脉高压。患者3为特发性肺动脉高压。患者4为骨形成蛋白Ⅱ型受体(bone morphogenetic protein type Ⅱ receptor,BMPR2)相关性肺动脉高压。患者5及患者6分别为活化素Ⅰ型受体(activator type Ⅰ receptor,ACVR1)(c.789C>A,p.D263E)及ACVR1(c.1450C>T,p.R484W)相关性肺高压。结论心导管检查对于肺动脉高压诊断和治疗具有指导意义,特别是SPA对肺动脉高压分型具有一定诊断意义。Objectives To summarize the results of cardiac catheterization in patients with different types of pulmo⁃nary hypertension(PAH),and to analyze their characteristics,in order to provide references for clinical diagnosis and treatment.Methods The data of 6 patients with PAH were regressively analyzed,including clinical symptoms,cardiac ultrasound,cardiac catheterization data[the ratio of pulmonary pressure to aortic pressure,pulmonary vascular resis⁃tance index(PVRI),selective pulmonary arteriography(SPA),pulmonary circulation time(PCT),acute pulmonary vasodilation test(AVT),etc],as well as treatment effects and follow-up.Results Case 1 was suggested ventricular septal defect(VSD)with dynamic PAH.Case 2 was VSD with non-dynamic PAH.Case 3 was idiopathic PAH.Case 4 was bone morphogenetic protein typeⅡreceptor(BMPR2)-related PAH.Cases 5 and 6 were PAH associated with activator type I receptor(ACVR1)(c.789C>A,p.D263E)and ACVR1(c.1450C>T,p.R484W).Conclusions Cardiac catheterization has guiding significance for the diagnosis and treatment of PAH.Especially,SPA has certain diagnostic significance for the classification of PAH.

关 键 词：肺动脉高压 小儿 心导管术 

分 类 号：R543.2[医药卫生—心血管疾病]