Clinical classification of pulmonary hypertension

https://doi.org/10.1016/j.jacc.2004.02.037Get rights and content
Under an Elsevier user license
open archive

Abstract

In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term “primary pulmonary hypertension” and to replace it with “idiopathic pulmonary hypertension”; to reclassify pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for pulmonary arterial hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-pulmonary shunts.

Abbreviations

ALK1
activin-receptor-like kinase-1
APAH
pulmonary arterial hypertension related to risk factors or associated conditions
BMPR2
bone morphogenetic protein receptor type II
FPAH
familial pulmonary arterial hypertension
IPAH
idiopathic pulmonary arterial hypertension
PAH
pulmonary arterial hypertension
PCH
pulmonary capillary hemangiomatosis
PH
pulmonary hypertension
PPH
primary pulmonary hypertension
PVOD
pulmonary veno-occlusive disease
TGF-β
transforming growth factor-β

Cited by (0)