2026 年第 2 期 第 21 卷

非先天性心脏病儿童肺动脉高压病因分布及其预后分析

Distribution of etiologies and prognostic analysis of pulmonary hypertension in children without congenital heart disease

作者：刘倩李博

英文作者：Liu Qian Li Bo

单位：河北省儿童医院心内科河北省小儿心血管重点实验室河北省儿童健康与疾病临床医学研究中心，石家庄050000

英文单位：Department of Cardiology Hebei Children′s Hospital Hebei Provincial Key Laboratory of Pediatric Cardiovascular Disease Hebei Provincial Clinical Research Center for Child Health and Disease Shijiazhuang 050000 China

关键词：儿童肺动脉高压；病因分布；预后分析

英文关键词：Pediatricpulmonaryhypertension;Etiologicaldistribution;Prognosticanalysis

• 摘要：

• 目的 分析非先天性心脏病儿童肺动脉高压（PH）的病因分布及其预后。方法 选取2017年11月至2024年5月就诊于河北省儿童医院心内科年龄<14岁的非先天性心脏病相关PH患儿34例。根据病因进行分组，A组为第一大类动脉型PH，B组为第三大类肺部疾病和/或低氧所致PH，C组为第五大类未明原因或多因素所致PH。收集患儿临床信息，对其病因分布进行描述，绘制生存曲线分析生存率差异。结果 本研究共纳入34例患儿，其中动脉型PH 15例（A组）、肺部疾病和/或低氧相关性PH 14例（B组）、C组5例患儿，C组均为甲基丙二酸血症患儿。B组年龄、经皮血氧饱和度、肺动脉收缩压水平小于/低于A组，早产比例、动脉血二氧化碳分压水平均高于A组，差异均有统计学意义（均P＜0.05）。21例患儿因就诊时病情危重入重症监护室治疗，其余患儿病情相对平稳于普通病房治疗。A组1、3、5年生存率分别为72.7%、72.7%、54.5%，B组为49.0%、39.2%、39.2%，C组为100.0%、75.0%、75.0%。入重症监护室患儿1、3、5年生存率分别为55.7%、50.1%、37.6%，非重症监护室患儿1、3、5年生存率分别为100.0%、75.0%、56.3%，组间比较差异有统计学意义（Log-rank χ2=4.700，P=0.030）；2岁及以上患儿1、3、5年生存率分别为87.4%、77.7%、64.7%，2岁以下患儿1、3、5年生存率分别为58.2%、41.4%、41.4%，组间比较差异有统计学意义（Log-rank χ2=4.793，P＝0.029）。结论 儿童PH病因分布广泛，就诊时心功能差，部分患儿就诊时病情危重需重症监护治疗，且预后欠佳。肺部疾病或低氧相关PH、就诊时病情危重入重症监护室、诊断年龄小等和预后不良相关。

• Objective To analyze the etiological distribution and prognosis of pulmonary hypertension (PH) in children without congenital heart disease. Methods A total of 34 children with PH unrelated to congenital heart disease, aged <14 years, who were admitted to the Department of Cardiology, Hebei Children′s Hospital from November 2017 to May 2024 were enrolled. According to the etiologies, the patients were divided into three groups: Group A (Group 1 pulmonary arterial hypertension), Group B (Group 3 PH due to lung diseases and/or hypoxia), and Group C (Group 5 PH due to unclear or multifactorial causes). Clinical data of the children were collected, the etiological distribution was described, and survival curves were plotted to analyze the differences in survival rates. Results A total of 34 children were enrolled in this study, including 15 cases of arterial PH (Group A), 14 cases of lung disease and/or hypoxia-related PH (Group B), and 5 cases in Group C, all of whom were diagnosed with methylmalonic acidemia. In Group B, the age, transcutaneous oxygen saturation, and pulmonary artery systolic pressure were lower than those in Group A, while the proportion of preterm infants and arterial partial pressure of carbon dioxide level were higher than those in Group A (all P<0.05). Twenty-one children were admitted to the intensive care unit for treatment due to critical conditions at admission, while the remaining children with relatively stable conditions were treated in general wards. The 1-year, 3-year, and 5-year survival rates were 72.7%, 72.7%, and 54.5% in Group A, 49.0%, 39.2%, and 39.2% in Group B, and 100.0%, 75.0%, and 75.0% in Group C, respectively. The 1-year, 3-year, and 5-year survival rates of children admitted to the intensive care unit were 55.7%, 50.1%, and 37.6%, respectively, while those of children not admitted to the intensive care unit were 100.0%, 75.0%, and 56.3%, with a statistically significant difference between the two groups (Log-rank χ2=4.700, P=0.030). For children aged 2 years and above, the 1-year, 3-year, and 5-year survival rates were 87.4%, 77.7%, and 64.7%, respectively, compared with 58.2%, 41.4%, and 41.4% for children under 2 years of age, and the difference between the groups was statistically significant (Log-rank χ2=4.793, P=0.029). Conclusions The etiologies of PH in children are widely distributed. Children with PH often present with poor cardiac function at admission; some are critically ill and require intensive care, with an unfavorable prognosis. Lung disease or hypoxia-related PH, critical condition requiring intensive care unit admission at presentation, and young age at diagnosis are associated with poor prognosis.