诊断性治疗左向右分流型先天性心脏病合并重度肺动脉高压中靶向药物的应用及手术适应证的选择  

Application of targeted drugs in diagnostic treatment of severe pulmonary hypertension associated with left-to-right shunt congenital heart disease and its values in surgical indication selection

作　　者：王执一 景小勇[1] 杨学勇[1] 李刚[1] 苏俊武[1] 刘迎龙[1] 范祥明[1] Wang Zhiyi,Jing Xiaoyong, Yang Xueyong, Li Gang, Su Junwu, Liu Yinglong, Fan Xiangming(Pediatric Cardiac Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, Chin)

机构地区：[1]首都医科大学附属北京安贞医院小儿心脏中心,100029

出　　处：《中国医药》2018年第5期696-700,共5页China Medicine

基　　金：国家自然科学基金(81600383);北京市卫生系统高层次卫生技术人才培养计划(2015-3-051)~~

摘　　要：目的评估靶向药物在诊断性治疗左向右分流型先天性心脏病合并重度肺动脉高压患者手术适应证选择中的临床价值。方法回顾性分析2011年1月至2017年6月首都医科大学附属北京安贞医院收治的左向右分流型先天性心脏病合并重度肺动脉高压患者55例。在强心利尿药物治疗的基础上,联合应用波生坦诊断性治疗,并通过右心导管检查、超声心动图和经皮血氧饱和度(SpO_2)改善情况评估波生坦治疗效果欠佳患者,联合应用皮下/静脉输注前列环素类药物(曲前列尼尔)治疗后,再次评估是否符合手术指征,对符合手术指征患者行外科手术根治,对术后残存肺动脉高压和不符合手术指征患者长期联合控制肺动脉高压药物治疗并进行远期超声心动图随访。结果本研究中单用波生坦患者32例(58.2%),治疗后SpO_2水平由86%～94%[(91.5±2.2)%]上升至95%～99%[(97.1±1.7)%]。序贯联合靶向药物治疗患者23例(41.8%),经联合用药后12例患者SpO_2水平由70%～94%[(89.4±8.2)%]上升至95%～99%[(96.6±2.1)%];4例复杂畸形患者SpO_2水平由68%～79%[(72.1±4.3)%]上升至82%～91%[(86.0±6.0)%],平均上升水平(17.7±3.4)%;另有7例患者SpO_2水平用药后仍维持在78%～92%[(89.1±5.3)%],目前继续靶向药物治疗。经诊断性治疗后48例(87.3%)患者行外科根治手术治疗,无围术期死亡,术后恢复良好;7例(12.7%)患者未达手术指征继续靶向药物治疗。全组手术患者随访3～6个月,6例患者肺动脉压力恢复正常,37例患者降至轻中度肺动脉高压,5例患者术后残存重度肺动脉高压。结论诊断性治疗通过强心利尿药物加上靶向药物的联合治疗,对传统意义上不符合手术指征的患者提供了可能根治手术的机会,同时对术后残存肺动脉高压和不符合手术指征的患者降低肺动脉压力、改善生活质量也有着重要意义。Objective To evaluate the value of targeted drugs in diagnostic treatment and surgical indication selection for left-to-rlght shunt congenital heart disease patients with severe pulmonary hypertension. Methods Clinical data of 55 left-to-right shunt congenital heart disease patients with severe pulmonary hypertension were reviewed from January 2011 to June 2017 in Beijing Anzhen Hospital, Capital Medical University. All patients were treated with bosentan on the basis of eardiotonic and diuretic agents; patients with no significant response to bosentan had subcutaneous/intravenous infusion of prostacyclin drug （treprostinil）. Patients who showed surgical indications after diagnostic treatment had radical operation; patients without surgical indications and patients with residual pulmonary hypertension after operation were treated with long-term drug therapy and had regular echocardiography reexamination. Results Thirty-two patients （58.2%） had bosetan monotherapy; the level of SpO2 increased from 86% -94% [ （91.5± 2.2 ） % ] to 95% -99% [ （97. 1 ± 1.7 ） % ] after treatment. Twenty-three patients （41.8%） had sequential targeted drug therapy; the level of SpO2 in 12 patients increased from 70%-94% [ （89.4 ± 8.2） % ] to 95% -99% [ （96. 6± 2.1 ） % ] after treatment ; the level of SpO2 in 4 patients with complex congenital heart disease increased from 68% -79% [ （72, 1 ±4.3 ）% ] to 82% -91% [ （86.0 ±6. 0）% ] and the average riselevel was （ 17.7 ±3.4 ） % ; the level of SpO： in 7 patients remained at 78% -92% [ （ 89. 1 ± 5.3 ） % ] after treatment. Forty-eight patients（87.3% ） had surgical radical operation after diagnostic treatment without perioperative death and recovered well; 7 patients（ 12.7% ） continued to be treated with target drugs. All patients were followed up for 3-6 months; pulmonary artery pressure recovered to normal in 6 patients; 37 patients recovered to mild- to-moderate pulmonary hypertension; 5 patients had residual severe pulmonary hyperte

关 键 词：先天性心脏病 肺动脉高压 靶向药物 诊断性治疗 

分 类 号：R541[医药卫生—心血管疾病]