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J Am Coll Cardiol, 2004; 43:5-12 © 2004 by the American College of Cardiology Foundation |









* Department of Pulmonary and Critical Medicine, University of Paris Sud, Paris, France
Institute of Cardiology, University of Bologna, Bologna, Italy
Division of Pulmonary and Critical Care Medicine, University of California, San Diego, California, USA
Department of Medicine, Sir Mortimer B. Davis Jewish General Hospital, McGill University, Montreal, Canada
|| Department of Internal Medicine II, Justus-Liebig-University, Giessen, Germany
¶ Department of Intensive Care and Pneumology, Regional Hospital of Locarno, Locarno, Switzerland
National Heart and Lung Institute, Imperial College of Science, Technology and Medicine, London, United Kingdom
** Department of Hepatology, INSERM U481, Beaujon Hospital, Clichy, France

Department of Internal Medicine, University Hospital of Zurich, Zurich, Switzerland

Pediatric Cardiology Unit, Children's University Hospital of Geneva, Geneva, Switzerland

Center for Pulmonary Heart Disease, Rush-Presbyterian-St. Luke's Medical Center, Chicago, Illinois, USA
|||| University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
Manuscript received January 28, 2004; revised manuscript received February 13, 2004, accepted February 23, 2004.
*
Reprint requests and correspondence: Dr. Gerald Simonneau, Department of Pneumology and Intensive Care Unit, Hôpital Antoine Béclère, 157 rue de la Porte de Trivaux, 92141 Clamart, France.
gerald.simonneau{at}abc.ap-hop-paris.fr
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.
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