肺动脉高压(pulmonary arterial hypertension,PAH)是先天性心脏病(congenital heart disease,CHD)最常见的合并症,在各型CHD当中,肺动脉高压的诊断和治疗贯穿术前、术中和术后,甚至伴随患者终生。文章将从流行病学、分类、筛查与诊断、血流动力学评价,以及靶向治疗带来的新机会和预后的变化等几个方面,阐述CHD相关PAH近几年的进展。 Pulmonary arterial hypertension (PAH) is the most common comorbidity of congenital heart disease (CHD). Among various types of CHD, pulmonary hypertension (PAH) is diagnosed and treated preoperatively, intraoperatively and postoperatively, and even With the patient’s life. In this article, the progress of CHD-related PAH in recent years will be described in terms of epidemiology, classification, screening and diagnosis, hemodynamic evaluation, and new opportunities and prognostic changes brought about by targeted therapy.