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

Gerald Simonneau, MD*,*, Nazzareno Galiè, MD{dagger}, Lewis J. Rubin, MD{ddagger}, David Langleben, MD§, Werner Seeger, MD||, Guido Domenighetti, MD, Simon Gibbs, MD, Didier Lebrec, MD**, Rudolf Speich, MD{dagger}{dagger}, Maurice Beghetti, MD{ddagger}{ddagger}, Stuart Rich, MD§§ and Alfred Fishman, MD||||

* Department of Pulmonary and Critical Medicine, University of Paris Sud, Paris, France
{dagger} Institute of Cardiology, University of Bologna, Bologna, Italy
{ddagger} 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
{dagger}{dagger} Department of Internal Medicine, University Hospital of Zurich, Zurich, Switzerland
{ddagger}{ddagger} 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.

Abbreviations and Acronyms
  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-ß = transforming growth factor-ß




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