波生坦用于儿童先天性心脏病相关肺动脉高压66例临床评价

目的探讨波生坦治疗儿童先天性心脏病（CHD）相关肺动脉高压（PAH）的临床疗效。方法选取医院2012年至2013年收治的66例CHD患儿,其中左向右分流组（A组）38例,功能性单心室（FSV）组（B组）28例,均口服波生坦治疗,随访并评估临床疗效与不良反应。结果 A组患儿治疗1-3个月后肺动脉高压均明显下降,心功能明显改善,差异有统计学意义（P〈0.05）。B组患儿治疗前心功能Ⅳ,Ⅲ,Ⅱ级构成比分别为32.14%（9/28）,46.43%（13/28）,21.43%（6/28）,末次随访各级构成比分别为0,21.43%（6/28）,71.43%（20/28）,另2例（7.14%）为心功能Ⅰ级,差异均有统计学意义（P〈0.05）;治疗前脉搏血氧饱和度（Sp O2）为（79±5）%,末次随访时Sp O2为（85±5）%,差异有统计学意义（P〈0.05）;治疗前B组患者颜面部水肿14例、胸腔积液15例、心包积液9例,末次随访时颜面部水肿2例、胸腔和心包积液各1例,差异有统计学意义（P〈0.05）。结论波生坦治疗儿童CHD相关PAH疗效良好,值得临床推广。

Clinical Evaluation on Application of Non Selective Endothelin Receptor Antagonist in 66 Cases of Child Congenital Heart Disease Associated PAH

Objective To investigate the clinical effect of bosentan in treating child congenital heart disease（CHD） associated pulmonary arterial hypertension（PAH）. Methods 66 children patients with CHD in our hospitals from 2012 to 2013 were selected as the research subjects,including 38 cases of left to right shunt（group A） and 28 cases of functional single ventricle（FSV,group B）,and treated by oral bosentan. The clinical effects and adverse reactions were followed up and evaluated. Results PAH after 1- 3 months therapy in the group A was decreased obviously and the cardiac function was significantly improved,the difference showed statistical significance（P〈0. 05）; the constituent ratio of heart function grade Ⅳ,Ⅲ,Ⅱ before treatment in the group B was 32. 14%（9/28）,46. 43%（13/28） and 21. 43%（6/28） respectively,the constituent ratio of various grades at last follow- up was 0,21. 43%（6/28） and71. 43%（20/28） respectively,other 2 cases（7. 14%） were the cardiac function grade Ⅰ,the differences were statistically significant（P〈0. 05）; pulse Sp O2 before therapy was（79 ± 5） %,which at last follow up was（85% ± 5%）,the difference had statistical significance（P〈0. 05）; before treatment,the group B had 14 cases of facial edema,15 cases of pleural effusion and 9 cases of pericardial effusion,but at last follow- up facial edema was in 2 cases,pleural effusion was in 1 case and pericardial effusion was in 1 case,the differences had statistical significance（P〈0. 05）. Conclusion Bosentan has better effect in treating child CHD associated PAH,which is worthy of clinical promotion.