In recent years, several important outcomes have allowed increasing our understanding in the biopathology of idiopathic pulmonary arterial hypertension. Although the triggering event remains unknown, some of the adjacent mechanisms of disease had been identified. Pharmacological manipulation of such mechanisms, due to advances in the biopathology, has produced some clear clinical benefits, related to the survival of these patients. However, little exploration has been done on other pathways such as apoptosis or gene manipulation, which may be of great interest both for scientific and clinical research.