先天性心脏病合并重度肺动脉高压诊断性治疗的临床研究  

Study of diagnostic treatment in patients suffering congenital heart disease combined with severe pulmonary arterial hypertension

作　　者：景小勇[1] 杨学勇[1] 苏俊武[1] 刘承虎[1] 陈哲[1] 顾虹[1] 刘迎龙[1] 

机构地区：[1]北京首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所小儿心脏科,100029

出　　处：《心肺血管病杂志》2016年第4期293-296,共4页Journal of Cardiovascular and Pulmonary Diseases

基　　金：国家自然科学基金资助项目(81570443);2011年北京市科技计划项目(Z111100074911001)

摘　　要：目的：评估诊断性治疗在选择伴艾森门格综合征的左向右分流型先天性心脏病（先心病）外科根治手术的临床价值。方法：回顾性分析2011年1月至2015年7月,行手术治疗的先天性心脏病合并重度肺动脉高压患者32例（男性8例,女性24例）。年龄9~52岁,中位年龄23岁,体质量25~60kg,平均（45.9±10）kg,其中房间隔缺损8例,室间隔缺损18例,部分型心内膜垫缺损2例,单纯动脉导管未闭2例,动脉导管未闭合并房间隔缺损、室间隔缺损各1例。首诊时经皮血氧饱和度（Sp O2）86%~94%,平均（91.5±2.2）%。均予强心利尿治疗的基础上,加用西地那非或波生坦等选择性降低肺动脉压靶向药物8周到24周,行右心导管检查后在全麻体外循环下行外科手术根治,术中放置4/5#SwanGanz漂浮导管监测术中及术后肺动脉压。术后残余肺动脉高压患者继续给予降低肺动脉压力药物治疗。术后1个月、3个月、6个月及以后每年超声心动图检查随访。结果：患者经药物靶向治疗后Sp O2均〉95%,平均（97±1.7）%,术前右心导管检查肺动脉平均压（m PAP）47~93mm Hg,平均[（75±13）mmHg,1mm Hg=0.133k Pa],肺血管阻力指数（PVRI）平均（10.2±3.1）Wood unit·m-2（4.6~15.3）,肺循环血流量与体循环血流量比值（Qp/Qs）1.30~2.98,平均（1.84±0.44）;导管检查中行急性肺血管扩张实验阳性31例,阴性1例。术后随访6个月~4年,全组术后至随诊无一例死亡,无远期并发症。结论：本组病例提示,诊断性靶向药物治疗作为慢性肺血管扩张实验,为错过良好手术时机的艾森门格综合征前期患者提供了可能根治的机会。术后残余肺动脉高压患者继续降低肺动脉压力药物治疗仍有一定疗效。Objective： This study is to evaluate the value of diagnostic treatment on identifying surgical indication for patients with left to right shunt congenital heart disease and Eisenmenger syndrome. Methods：This pulmonary arterial hypertension（ Gender： 8 male; 24 female; Age： 9-52 [median： 23]; Weight： 25-60kg（ 45. 9 ± 10） kg. They were surgically treated from 2011 January to 2015 July. The disease types included atrial septal defect（ 8 cases）,ventricular septal defect（ 18）,partial endocardial cushion defect（ 2）,simple patent ductus arteriosus（ 2）,patent ductus arteriosus combined with atrial septal defect（ 1） and patent ductus arteriosus with ventricular septal defect（ 1）. Patients＇ Sp O2 at initial diagnosis was（ 91. 5 ± 2. 2） %（ 86%-94%）. Patients received targeted therapies（ Sildenafil,Bosentan,etc.） to lower pulmonary artery pressure on the basis of cardiotonic agents and diuretic therapies for 8 to 24 weeks during pre-operation. Right cardiac catheterization was conducted and then surgical operation was performed under general anesthesia and extracorporeal circulation. A 4 /5#Swan-Ganz float catheter was used to monitor pulmonary arterial pressure during the operation and also to evaluate pressure change after surgery. Treatments for lowering pulmonary artery pressure were continued during post-operation if the condition was not improved. Ultrasound cardiogram was performed as follow-up on 1,3 and 6 months after surgery. Result： After the treatment,Sp O2 were above 95%（ 97 ± 1. 7） %in all patients. Cardiac catheterization showed mean pulmonary arterial pressure（ m PAP） was 47-93 mm Hg（ 75± 13） mm Hg（ 1 mm Hg = 0. 133 Kpa） and pulmonary vascular resistance index（ PVRI） was 4. 6-15. 3（ 10. 2± 3. 1） Wood unit·m2; the ratio of pulmonary to systemic flow（ Qp/Qs） was 1. 30-2. 98（ 1. 84 ± 0. 44）. Acute pulmonary vasodilator testing showed 31 positive and 1 negative. With a follow-up of 6 months- 4 years,all patients surviv

关 键 词：先天性心脏病 肺动脉高压 诊断性治疗 

分 类 号：R54[医药卫生—心血管疾病]