Author + information
- Received January 28, 2004
- Revision received February 13, 2004
- Accepted February 23, 2004
- Published online June 16, 2004.
- Gerald Simonneau, MD*,* (, )
- Nazzareno Galiè, MD†,
- Lewis J Rubin, MD‡,
- David Langleben, MD§,
- Werner Seeger, MD∥,
- Guido Domenighetti, MD¶,
- Simon Gibbs, MD#,
- Didier Lebrec, MD**,
- Rudolf Speich, MD††,
- Maurice Beghetti, MD‡‡,
- Stuart Rich, MD§§ and
- Alfred Fishman, MD∥∥
- ↵*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.
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.
- Received January 28, 2004.
- Revision received February 13, 2004.
- Accepted February 23, 2004.
- American College of Cardiology Foundation
- Evian classification
- Assessment of the Evian classification
- Do we need a genetic classification of PH?
- To abandon the term “primary pulmonary hypertension”
- To reclassify PVOD and PCH
- Updated risk factors and associated conditions for pulmonary arterial hypertension
- Recent epidemiologic studies
- Case series and case reports
- Classification of congenital systemic-to-pulmonary shunts