Pulmonary arterial hypertension (PAH) is a group of diseases related to progressively increasing pulmonary vascular resistance, a high incidence of right ventricular failure and premature death. Only a limited number of pharmaceutical therapies have proven to be beneficial for PAH. These therapies improve symptoms, exercise capacity, and haemodynamics; however, the clinical relevance of these effects has been challenged. Therefore, the effect of currently approved treatment options remains inconclusive. Conversely, several new drugs for various aetiologies and clinical stages are expected to provide significant advances for the treatment of PAH. Moreover, percutaneous pulmonary artery denervation treatment may lead to a new therapeutic orientation in patients with PAH. The aim of this review is to present the new developments in PAH treatment, provide a brief overview of future directions in the field and discuss the potential future prospects of these innovative therapies.