Fontan术后应用波生坦对中远期预后的影响

Effect of Bosentan on the long-term prognosis in Fontan patients

目的探讨波生坦对Fontan术后患者中远期预后的影响。方法符合条件的9例受试者被随机分为治疗组和安慰剂组。波生坦治疗的方案与肺动脉高压靶向药物治疗的方案一样，根据公斤体重使用药物剂量，每日2次。所有患者均于第2个月开始增加波生坦剂量至前1个月的2倍，服药时间〉1年。在手术后的第2年，所有患者均在我院行心脏CT检查，并进行心功能NYHA分级，检测6min步行距离（6MWT）和肝功能（A坍）。结果2年后揭盲，两组均无死亡病例。试验组在心功能和6MWT方面显著优于对照组，差异有统计学意义。术后2年复查心脏CT，发现两组患者均存在肺部下野细小肺动一静脉瘘生成的现象，但是都未观察到粗大肺动一静脉瘘，试验组发生率（20％）虽然小于对照组（50％），但是未见统计学差异。肝功能ALT两组比较未见统计学差异，初步确定了波生坦在本研究中的安全性。结论波生坦能够改善Fontan术后患者纽约心功能状况，增加6min步行距离，但没有观察到在其他方面的获益。

Objective To investigate the effects of Bosentan on the long-term prognosis of patients after Fontan operation. Methods Eligible 9 participants will be randomly divided into the treatment group and control group. The treatment programs of Bosentan was given same as the treatment programs of pulmonary arterial hyper- tension drug. In the second year after surgery, the dose of drug based on kg of body weight, twice a day. In the second months, all patients increase the dose of Bosentan 2 times to the previously, taking time more than a year. All patients took the examinations of cardiac CT examination, cardiac function NYHA class, 6 MWT and liver function （ALT） in our hospital. Results After 2 years, the trial was unblinding, two groups of patients had no death. But in the secondary indexes on two showed some differences, the cardiac function and 6 MWT in test group were better than control group, the difference has statistical significance. The review of cardiac CT after 2 years, found out the two groups patients both had some subjects who had the formation of small lung pulmonary ar- teriovenous fistula, but no thick pulmonary arteriovenous fistula was observed. Although the incidence in treatment group （20%） was less than the control group （50%）, the difference was not statistically significant. There was no significant difference in ALT liver function between the two groups, preliminary determined the safety of Bosentan in this study. Conclusion Bosentan can improve New York Heart Association functional status and increase 6 minutes walking distance of patients received Fontan surgery, but can not bring more benefit in other ways.