Severe pulmonary hypertensive diseases: a perspective

Abstract

Clinically "severe pulmonary hypertension" is a group of diseases. The nomenclature of pulmonary hypertensive disorders is confusing since terms like "primary", "idiopathic", "unexplained" and "plexogenic" are often used interchangeably. 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 and clinical association is proposed and a recommendation to reorder "Severe pulmonary hypertension" in an association-morphology-function context is provided. The purpose of this "perspective" is to stimulate a discussion which hopefully will lead to a change in the presently used nomenclature and classification of pulmonary hypertensive disorders.