Clinical classification of pulmonary hypertension.
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.
Simonneau
Gerald
G
Department of Pulmonary and Critical Medicine, University of Paris Sud, Paris, France. gerald.simonneau@abc.ap-hop-paris.fr
Galiè
Nazzareno
N
Rubin
Lewis J
LJ
Langleben
David
D
Seeger
Werner
W
Domenighetti
Guido
G
Gibbs
Simon
S
Lebrec
Didier
D
Speich
Rudolf
R
Beghetti
Maurice
M
Rich
Stuart
S
Fishman
Alfred
A
D020022:Genetic Predisposition to Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification* / Q000175:diagnosis / Q000453:epidemiology; D011668:Pulmonary Veno-Occlusive Disease / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology; D012307:Risk Factors