Pulmonary hypertension (PH) is a common phenomenon that may occur as a consequence of various diseases (e.g., heart failure, chronic lung diseases, and pulmonary embolism), as a distinct disease of the small pulmonary arterioles, or a combination of both. Independently from the origin, PH has important impact on patient´s symptoms and life expectancy. The establishment of an exact diagnosis and classification, as well as the understanding of the hemodynamic interrelations, provides the basis for often challenging treatment decisions. Recently, the 5th World Symposium on PH took place in Nice, France, where important standards and definitions were specified. Furthermore, the results of recent phase III trials have led to the approval of new targeted therapies. The most relevant developments including the rating of novel treatment options are summarized in this article.