Objective: To explore the influencing factors of efficacy of bosentan in the treatment of children with pulmonary arterial hypertension. Methods: Totally 68 children with pulmonary arterial hypertension admitted into our hospital from Jun. 2013 to Jun. 2016 were extracted for regular follow-up. The follow-up endpoint was the death of the child or the treatment of lung transplantation. Clinical data were collected

including age

gender

body mass index (BMI)

course of disease

physical examination

blood routine and cardiac catheterization results after treatment. Univariate analysis and multivariate analysis were performed to evaluate the correlation between various factors and the therapeutic effects of bosentan on pulmonary arterial hypertension in children. Results: Age

edema

New York Heart Association (NYHA) grade of cardiac function

troponin I (TnI)

hemoglobin (HGB)

total bilirubin (TBil)

pulmonary vascular resistance index (PVRI) and other factors were correlated with the clinical effects of bosentan on pulmonary arterial hypertension in children (P<0.05). Further multivariate logistic regression analysis showed that age <5 years

NYHA grade of cardiac function from Ⅲ to Ⅳ

and TBil ≥6.23 µmol/L are independent risk factors for the poor efficacy (endpoint events) of bosentan on pulmonary arterial hypertension in children (P<0.05). Conclusion: There are many factors affecting the efficacy of bosentan in the treatment of pulmonary arterial hypertension in children

and targeted interventions should be taken in clinic.