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

Liu Qian; Li Bo

doi:10.3760/j.issn.1673-4777.2026.02.006

Abstract：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 pulmo-nary 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.