波生坦治疗先天性体-肺分流性心脏病患者肺动脉高压效果观察

目的观察波生坦治疗先天性体-肺分流性心脏病肺动脉高压患者肺动脉压的变化及封堵术后的疗效和安全性.方法选择昆明医学院附属延安医院心内科2009年7月至2009年12月行封堵术的先天性体-肺分流性心脏病肺动脉高压（PAPm≥25mmHg）患者60例为研究对象,遵循随机原则,治疗组（术前、后口服波生坦）30例,与对照组（术前、后未口服任何降低肺动脉压药物）30例,口服波生坦满6月后（术前3个月及术后3个月）观察治疗组和对照组WHO心功能分级,右室收缩压,6min步行试验（6WMD）的变化,肝损害的发生率.结果与对照组比较,治疗组患者：（1）6MWD增加有统计学意义（P〈0.01）;（2）右室收缩压改善率的比数比（OR）值有统计学意义（P〈0.05）;（3）WHO心功能分级改善率的OR值为2.18（95%CI为1.06～3.1,P〈0.05）;（4）肝损害的发生率差异无统计学意义,其合并OR值为1.72（95%CI为0.86～4.61,P〈0.05）.结论波生坦治疗可以明显降低肺动脉压和改善心功能,提高先天性体-肺分流性心脏病肺动脉高压患者封堵术后的运动耐力,可明显缓解病情恶化.肝损害可能是其主要副作用。

The Efficacy of Oral Bosentan before and after the Closure on Patients with Congenital Heart Disease and Pulmonary Shunt Induced Pulmonary Arterial Hypertension

Objective To observe the change of pulmonary arterial hypertension and the efficiency and safety after the closure on patients with congenital heart disease and pulmonary shunt induced pulmonary arterial hypertension（PAH） with the treatment of bosentan.Methods Sixty patients with congenital heart disease and pulmonary shunt induced PAH（PAPm ≥25 mmHg） who underwent the closure from July 2009 to December 2009 in our hospital were studied.The patients were randomly divied into two groups（30 cases in each group）：the treatment group（oral bosentan before and after the closure） and the control group（without any drugs to decrease the pulmonary arterial pressure before and after the closure）.After patients in the treatment group completed the 6 months of oral bosentan（3 months before and after the closure）,we compared the difference in WHO cardiac functional grading,right ventricular systolic pressure,6 Min walk test（6WMD） and the incidence of liver damage between the two groups.Results Compared with control group,the treatment group：（1） 6MWD increased significantly（P〈0.01）;（2） The improvement rate of odds ratio（OR） values in right ventricular systolic pressure was statistically significant（P 0.05）;（3） The improvement rate of OR values in WHO cardiac functional grading was 2.18（95% CI 1.06～3.1,P〈0.05）;（4） The incidence of liver damage had no significant statistical difference,the OR values was 1.72（95% CI 0.86～4.61,P〈0.05）.Conclusions Bosentan therapy can significantly reduce pulmonary arterial pressure,improve heart function,enhance exercise capacity and alleviate the disease progression of patients with congenital heart disease and pulmonary shunt PAH after the closure.Liver damage may be the major side effect.