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目的探讨法舒地尔治疗先天性心脏病相关性重度肺动脉高压短期应用的有效性及安全性。方法入选2011年1月~2012年8月我科住院的先天性心脏病相关性重度肺动脉高压患者,法舒地尔60 mg静脉滴注,每天2次,连续用药14 d后观察6分钟步行距离、血流动力学参数、超声心动图指标以及血浆N末端脑钠尿肽前体(NTpro BNP)变化情况。结果共纳入21(男4,女17)例患者,年龄16~54(32±14)岁;用药14 d后6分钟步行距离由(445±49)m显著增加至(471±40)m,差异有统计学意义;且WHO肺动脉高压心功能分级显著改善(P<0.05)。血浆NT-pro BNP亦明显下降,从(788±623)pg/ml降低至(464±393)pg/ml(P<0.05)。右房平均压从(6.1±2.3)mm Hg降低至(5.2±1.8)mm Hg(P<0.05);右向左分流量从(23±16)%明显减少至(18±16)%(P<0.05);而肺动脉平均压、肺血管阻力、体肺循环血流量比值、体肺循环阻力比值、股动脉氧饱和度、心脏指数等指标虽无显著统计学差异,但较用药前均有所改善,仅体循环压力及阻力均无统计学差异。结论法舒地尔短期内应用可显著提高先天性心脏病相关重度肺动脉高压患者的运动耐量、WHO肺动脉高压心功能分级,有效改善肺动脉高压的血流动力学指标,而且安全性与耐受性良好。
Objective To investigate the efficacy and safety of fasudil in short-term treatment of congenital heart disease associated with severe pulmonary hypertension. Methods January 2012 ~ August 2012 in our department of congenital heart disease associated with severe pulmonary hypertension, fasudil 60 mg intravenously, 2 times a day for 14 days after continuous administration observed 6 minutes walking distance , Hemodynamic parameters, echocardiographic parameters and plasma N-terminal pro-brain natriuretic peptide (NTpro BNP) changes. Results A total of 21 (male 4, female 17) patients aged 16- 54 years (32 ± 14) years were included in the study. The walking distance from 445 ± 49 m to 471 ± 40 m at 14 min after drug administration was significantly increased The difference was statistically significant; and WHO pulmonary heart function classification significantly improved (P <0.05). Plasma NT-pro BNP was also significantly decreased from (788 ± 623) pg / ml to (464 ± 393) pg / ml (P <0.05). Right atrium mean pressure decreased from (6.1 ± 2.3) mm Hg to (5.2 ± 1.8) mm Hg (P <0.05), and right to left shunt decreased from 23 ± 16% to 18 ± 16% <0.05). Although there was no significant difference between mean pulmonary arterial pressure, pulmonary vascular resistance, pulmonary blood flow rate, pulmonary resistance, femoral arterial oxygen saturation and cardiac index, they were all better than those before treatment, Only systemic pressure and resistance were not statistically different. Conclusion Fasudil can improve the exercise tolerance of patients with congenital heart disease associated with severe pulmonary hypertension in short-term, and classify the heart function of pulmonary hypertension in patients with pulmonary hypertension, which can effectively improve the hemodynamic indexes of pulmonary hypertension with good safety and tolerability .