Congenital heart disease complicated with pulmonary arterial hypertension(CHD-PAH)is an important type of pediatric pulmonary arterial hypertension,which affects the right heart function and can lead to right ventricular failure,and is the main cause of death in patients with CHD-PAH.Early prediction of the poor prognosis in children with CHD-PAH is essential for the positive changes in the appropriate treatment strategies,then improvement of the survival.In recent years,emerging biomarkers such as connective tissue growth factor,copeptin,endostatin,and circular RNA are predictive of clinical outcomes and prognosis in CHD-PAH patients and important for early and accurate identification of high-risk patients.This article discusses the correlation between the above novel biomarkers and the severity,disease progression and clinical outcome of CHD-PAH to provide a theoretical basis for physicians to utilize the relevant biomarkers in clinical diagnosis and treatment.