Pulmonary arterial hypertension (PAH) is a rare disease characterized by pulmonary vascular remodeling leading to increased vascular resistance. The increased afterload contributes to the development of right ventricular dysfunction and heart failure, which is the leading cause of death among patients with PAH. The development of specific treatments has markedly improved the prognosis of this population. However, PAH continues to be an incurable, life‑limiting condition, which creates a major burden for healthcare systems. This review describes the currently used treatments for PAH and provides insight into novel therapeutic targets that aim to reduce vascular remodeling, which ultimately leads to right ventricular failure.