波生坦治疗先天性心脏病相关性肺动脉高压临床疗效

The effect of bosentan in treatment of pulmonary arterial hypertension associated with congenital heart disease

目的研究本中心先天性心脏病相关性肺动脉高压患者服用波生坦治疗后,在降低肺动脉压力和改善运动耐力方面的效果,并验证服药耐受性。方法选择先天性心脏病相关性肺动脉高压患者42例,所有患者均口服波生坦治疗,在服药前、服药后每隔3月随访记录患者心功能分级、6分钟步行距离(6MWD)、超声心动图、实验室检查等信息,服药前及服药后不同时间点比较采用重复测量资料的方差分析,计数资料组间率(%)的比较采用x^2检验。结果 42例患者无失访,死亡1例,与服药前相比,随访12月后,患者具有较好耐受性,肺动脉压力从服药前(99.59±13.90)mm Hg下降为(53.80±10.95)mm Hg,6MWD从服药前时的(365.71±41.22)m增至(448.39±39.67)m,运动耐量提高。结论波生坦治疗可降低先心病相关性肺动脉高压患者肺动脉压力、提高运动耐力且有较好耐受性。

Objective To research the efficacy,safety of bostentan in patients with congenital heart disease(CHD)relate to pulmonary arterial hypertension(PAH)in our heart centre.Methods Totally,42pulmonary arterial hypertension associated with congenital heart disease patients were included in this single-center analysis.The patients who were enrolled in this clinical study were evaluated at baseline and every3months when follow-up by means of the New York Heart Association(NYHA)function class,six-minute walk distance(6-MWD),cardiopulmonary haemodynamic data and laboratory tests.Survival,assessed from starting bosentan to the end of the study,was summarized using the analysis of variance.The Chisquare test was used to compare variables between groups.Results42cases of patients without loss of follow-up except1cases of death.Compared with before treatment,patients with good tolerance,pulmonary artery pressure decreased from(99.59±13.90)mmHg to(53.80±10.95)mmHg.The6MWD increased from(365.71±41.22)m at baseline to(448.39±39.67)m and exercise tolerance increased when follow-up after December.Conclusions Negative selection endothelin receptor antagonist bostentan therapy is well safety,tolerated and significantly improve exercise capacity,haemodynamics in patients with PAH-CHD.