Abstract: Due to the high rates of mortality and morbidity of patients with congenital heart disease (CHD) with pulmonary arterial hypertension (PAH), the epidemiological characteristics and classification of CHD with PAH were reassessed by many CHD experts. In recent years, some new treatments and screening biomarkers, which may be used to reflect the severity and outcome endpoint of CHD patients with PAH, have been further explored. These results offer the possibility of improving diagnosis and treatment of CHD with PAH.