Pulmonary arterial hypertension(PAH) is the most common complication of congenital heart disease(CHD). Because of the complexity and diversity of cardiac anatomy and pathophysiology, as well as a variety of adaptive mechanisms that are not fully understood, little attention is paid on pulmonary arterial hypertension associated with congenital heart disease(CHD-PAH). This article aims to summarize the clinical status, disease characteristics, outcomes, and prognosis of CHD-PAH in Pediatric Cardiology, Beijing Anzhen Hospital, proposing current unresolved issues and future challenges.