Pulmonary hypertension (PH) is a severe disease characterized by pulmonary vascular remodeling and functional impairment, ultimately leading to right heart failure and high mortality rates. Ion channels, particularly calcium, potassium, and sodium channels, play crucial roles in the pathogenesis of PH. These ion channels regulate the activity of smooth muscle cells through various pathways, influencing vascular contraction, cell proliferation, and inflammatory responses. The dysregulation of these physiological and pathological processes plays a pivotal role in the onset and progression of PH. This article aims to comprehensively review the role and mechanisms of ion channels in the pathogenesis of PH, explore their potential as therapeutic targets, and provide a theoretical basis for developing novel treatment strategies for PH.