Abstract: Objective　To observe and analyze the etiology of pulmonary hypertension in single-center children and the hospitalization outcomes and influencing factors of patients with different types of pulmonary hypertension. Methods　A total of 1157 children with pulmonary hypertension diagnosed and treated in our hospital from January 2016 to December 2018 were selected. According to the inpatient data and discharge diagnosis，the etiology of pulmonary hypertension was classified according to the Expert Consensus on Diagnosis and Treatment of Pulmonary Artery Hypertension in Children issued in 2015 in China. Collect and record relevant clinical data during hospitalization. Results　A total of 1099 pulmonary artery hypertension（PAH） patients were involved in this study（94.9%），of which APAH-CHD and PPHN were the main types，accounting for 84.7% and 8.0%，respectively. Of the patients with idiopathic PAH，3 patients died，6 patients gave up treatment，1 patient underwent surgery，and the remaining 5 patients were improved and discharged. Of the patients with APAH-CHD，a total of 676 patients were discharged after surgery，accounting for 68.9%. Totally 45 patients asked to be discharged，accounting for 4.6%，of which 80 abandoned treatment or died，accounting for 8.2%. A total of 52 patients took bosentan，and 2 of 52 patients took sildenafil. Six patients with PPHN were discharged from the hospital after surgery for other organs，accounting for 6.5%，and 66 patients were improved and discharged，accounting for 70.9%；14 patients were discharged or died，accounting for 15.1%. In patients with left heart disease-related PH，18 cases was improved after treatment，11 cases was discharged after surgery，accounting for 40.7%，and 7 cases gave up or died，accounting for 25.9%. In respiratory disease-related PH，1 case died，and 3 cases gave up treatment. In the patients with PH caused by unknown factors，there were 2 patients giving up treatment，1 case died，and 1 patient was discharged after palliative surgery. Conclusion　Pulmonary hypertension in children is mainly APAH-CHD and PPHN. The children with idiopathic PAH or left heart disease-related PH have a poor prognosis.

Key words: child, classification of pulmonary hypertension, discharge outcome, treatment

摘要： 目的　观察分析单中心儿童肺高血压（PH）病因组成和不同类型PH患儿住院转归及影响因素。方法　选取2016年1月至2018年12月在湖南省儿童医院确诊并接受治疗的PH患儿共1157例，根据患儿病历资料及出院诊断，对其PH病因按照2015年发布的《儿童肺动脉高压诊断与治疗专家共识》进行分类。收集并记录其住院期间相关临床资料。结果　本研究中肺动脉高压（PAH）患儿共1099例（94.9%），以先天性心脏病相关性PAH（APAH-CHD）、新生儿持续肺动脉高压（PPHN）为主，分别占84.7%、8.0%。特发性PAH死亡3例，放弃治疗6例，手术1例，余5例好转出院。APAH-CHD中以手术后出院676例（68.9%）。要求出院45例（4.6%），其中放弃治疗出院及死亡80例（8.2%）；服用波生坦共52例，有2例联用西地那非。PPHN患儿因其他器官手术后出院6例（6.5%），好转出院66例（70.9%），放弃出院及死亡14例（15.1%）。左心疾病所致PH好转18例，行左心疾病矫治手术11例（40.7%），放弃及死亡病例7例（25.9%）。呼吸系统疾病PH 27例，1例死亡，3例放弃治疗。未知因素所致PH 2例放弃出院，死亡1例，1例行姑息手术出院。结论　儿童PH分类以APAH-CHD及PPHN为主，特发性PAH、左心疾病相关PH的患儿预后差。

关键词: 儿童, 肺高血压分类, 出院结局, 治疗