Clinical classification of pulmonary hypertension

Gerald Simonneau*, Nazzareno Galiè, Lewis J. Rubin, David Langleben, Werner Seeger, Guido Domenighetti, Simon Gibbs, Didier Lebrec, Rudolf Speich, Maurice Beghetti, Stuart Rich, Alfred Fishman

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1459 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)S5-S12
JournalJournal of the American College of Cardiology
Volume43
Issue number12 SUPPL.
DOIs
StatePublished - Jun 16 2004

Keywords

  • ALK1
  • APAH
  • BMPR2
  • FPAH
  • IPAH
  • activin-receptor-like kinase-1
  • bone morphogenetic protein receptor type II
  • familial pulmonary arterial hypertension
  • pulmonary arterial hypertension related to risk factors or associated conditions

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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