ABSTRACT
Originally known as primary pulmonary hypertension (PPH), idiopathic pulmonary arterial hypertension (IPAH) was first described by Dresdale et al. in 1951 (1). Three years later, the same authors described the occurrence of pulmonary hypertension among several members of the same family, providing the first report of what until recently has been known as familial pulmonary arterial hypertension (FPAH) (2). It is now recognized that PAH comes in many forms, including idiopathic (IPAH), hereditable (HPAH), and associated with drug exposures (such as anorectic fenfluramine compounds) or other medical conditions (3).
