Over the last decades, significant progress has been achieved in the pulmonary hypertension (PH) field. Pathophysiology of PH has been studied, leading to the classification of PH patients into five groups, while the hemodynamic definition has been recently revised. A diagnostic algorithm has been established and awareness has been raised in order to minimize diagnosis delay. The pulmonary arterial hypertension (PAH) treatment strategy includes the established three pathways of endothelin, nitric oxide-phosphodiesterase inhibitor, and prostacyclin pathway, but new therapeutic options are now being tested. The aim of this review is to summarize the existing practice and to highlight the novelties in the field of PH.