Severe pulmonary hypertensive diseases: a perspective

Clinically "severe pulmonary hypertension" is a group of diseases. The nome nclature of pulmonary hypertensive disorders is confusing since terms like "primary", "idiopathic", "unexplained" and "plexogenic" are often used inte rchangeably. In this Point of view or perspective the authors challenge the validity of the traditional pathohistological classification as it had been provided by HEATH and EDWARDS [1]. A classification that is focused on histological an d clinical association is proposed and a recommendation to reorder "Severe pulmonary hypertension" in an association-morphology-function context is pr ovided. The purpose of this "perspective" is to stimulate a discussion which hopefu lly mill lead to a change in the presently used nomenclature and classifica tion of pulmonary hypertensive disorders.