肺动脉高压(pulmonary arterial hypertension,PAH)是由多种病因引起的复杂疾病,发病机制尚未完全明了,其中先天性心脏病合并肺动脉高压的病人在临床上占相当大的比例,最终会导致心功能衰竭甚至死亡。随着医学的进步,肺动脉高压的治疗也在不断完善。本文通过复习文献,对目前肺动脉高压的诊断及治疗进行综述。 Pulmonary arterial hypertension (PAH) is a complex disease caused by a variety of etiologies. The pathogenesis of PAH is not yet fully understood. Clinically, patients with congenital heart disease complicated with pulmonary hypertension account for a large proportion and eventually lead to heart failure even death. With the progress of medicine, the treatment of pulmonary hypertension is also constantly improving. This article reviews the literature review of the current diagnosis and treatment of pulmonary hypertension.