Abstract：Objective To explore the effect of Bosentan combined with Sildenafil in the treatment of congenital heart disease（CHD）with severe pulmonary arterial hypertension（PAH）.Methods 61 patients diagnosed with CHD with severe PAH from September 2012 to December 2015 in our hospitalwere selected and divided into the control group（30 cases）and the treatment group （31 cases）according to the random number tablemethod.All the patients were treated with routine treatment，the control group was treated with Sildenafil on the basis of routine treatment，the treatment group was treated with Bosentan on the basis of the control group.The level of brain natriuretic peptide （BNP），dyspnea score （Borg score），oxygen saturation （SpO2）level，the grade of cardiac function classification and the improvement of right heart catheterization results before and after treatment in the two groups was compared.Results The level of brain natriuretic peptide （BNP），Borg score，NYHAFC score after treatment in the treatment group was lower than that before treatment and the control group after treatment，the level of SpO2after treatment in the treatment group was higher than that before treatment and the control group after treatment，with significant difference（P＜0.05）.The level of Pp/Ps，sPAP，mPAP after treatment in the treatment group was lower than that before treatment and the control group after treatment，the level of Qp/Qs after treatment in the treatment group was higher than that before treatment and the control group after treatment，with significant difference （P＜0.05）.Conclusion The effect of Bosentan combined with Sildenafil in the treatment of CHD with SPH is significant，which can improve the cardiopulmonary function better，with higher qualified life level，it isworthy of clinical promotion and application.
艾凌云 ；郭晨； 丁琰； 陈霞. 波生坦联合西地那非治疗先天性心脏病合并重度肺动脉高压的临床观察[J]. 中国当代医药, 2016, 23(26): 112-114.
AI ling-Yun； GUO Chen； DING Yan； CHEN Xia. Clinical observation of Bosentan combined with Sildenafil in the treatment of congenital heart disease with severe pulmonary arterial hypertension. 中国当代医药, 2016, 23(26): 112-114.
ApitzC，Reyes JT，Holtby H，etal.Pharmacokinetic and hemodynamic responses to oral sildenafil during invasive testing in children with pulmonary hypertension［J］.JAm Coll Cardiol，2010，55（14）：1456-1462．
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