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Clinical classification of pulmonary hypertension

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^||||

^* 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.

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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				L. Ray, M. Mathieu, P. Jespers, I. Hadad, M. Mahmoudabady, A. Pensis, S. Motte, I. R. Peters, R. Naeije, and K. McEntee Early increase in pulmonary vascular reactivity with overexpression of endothelin-1 and vascular endothelial growth factor in canine experimental heart failure Exp Physiol, March 1, 2008; 93(3): 434 - 442. [Abstract] [Full Text] [PDF]

				K. Asosingh, M. A. Aldred, A. Vasanji, J. Drazba, J. Sharp, C. Farver, S. A.A. Comhair, W. Xu, L. Licina, L. Huang, et al. Circulating Angiogenic Precursors in Idiopathic Pulmonary Arterial Hypertension Am. J. Pathol., March 1, 2008; 172(3): 615 - 627. [Abstract] [Full Text] [PDF]

				National Pulmonary Hypertension Centres of the UK Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland Thorax, March 1, 2008; 63(Suppl_2): ii1 - ii41. [Full Text] [PDF]

				J. M. Golbin, V. K. Somers, and S. M. Caples Obstructive Sleep Apnea, Cardiovascular Disease, and Pulmonary Hypertension Proceedings of the ATS, February 15, 2008; 5(2): 200 - 206. [Abstract] [Full Text] [PDF]

				R. Faludi, A. Komocsi, J. Bozo, G. Kumanovics, L. Czirjak, L. Papp, and T. Simor Isolated diastolic dysfunction of right ventricle: stress-induced pulmonary hypertension Eur. Respir. J., February 1, 2008; 31(2): 475 - 476. [Full Text] [PDF]

				K. L. SWANSON and M. J. KROWKA Screen for portopulmonary hypertension, especially in liver transplant candidates Cleveland Clinic Journal of Medicine, February 1, 2008; 75(2): 121 - 136. [Abstract] [Full Text] [PDF]

				R. Souza, M. Humbert, B. Sztrymf, X. Jais, A. Yaici, J. Le Pavec, F. Parent, P. Herve, F. Soubrier, O. Sitbon, et al. Pulmonary arterial hypertension associated with fenfluramine exposure: report of 109 cases Eur. Respir. J., February 1, 2008; 31(2): 343 - 348. [Abstract] [Full Text] [PDF]

				B. Petersen, M. Deja, R. Bartholdy, B. Donaubauer, S. Laudi, R. C. E. Francis, W. Boemke, U. Kaisers, and T. Busch Inhalation of the ETA receptor antagonist LU-135252 selectively attenuates hypoxic pulmonary vasoconstriction Am J Physiol Regulatory Integrative Comp Physiol, February 1, 2008; 294(2): R601 - R605. [Abstract] [Full Text] [PDF]

				C. F. Barnett, P. Y. Hsue, and R. F. Machado Pulmonary Hypertension: An Increasingly Recognized Complication of Hereditary Hemolytic Anemias and HIV Infection JAMA, January 23, 2008; 299(3): 324 - 331. [Abstract] [Full Text] [PDF]

				A. G. Lorenzoni, N. B. Anthony, and R. F. Wideman Jr. Transpulmonary Pressure Gradient Verifies Pulmonary Hypertension is Initiated by Increased Arterial Resistance in Broilers Poult. Sci., January 1, 2008; 87(1): 125 - 132. [Abstract] [Full Text] [PDF]

				O. Sitbon, C. Lascoux-Combe, J.-F. Delfraissy, P. G. Yeni, F. Raffi, D. De Zuttere, V. Gressin, P. Clerson, D. Sereni, and G. Simonneau Prevalence of HIV-related Pulmonary Arterial Hypertension in the Current Antiretroviral Therapy Era Am. J. Respir. Crit. Care Med., January 1, 2008; 177(1): 108 - 113. [Abstract] [Full Text] [PDF]

				M. K. Han, V. V. McLaughlin, G. J. Criner, and F. J. Martinez Pulmonary Diseases and the Heart Circulation, December 18, 2007; 116(25): 2992 - 3005. [Abstract] [Full Text] [PDF]

				G. D. Williams, B. M. Philip, L. F. Chu, M. G. Boltz, K. Kamra, H. Terwey, G. B. Hammer, S. B. Perry, J. A. Feinstein, and C. Ramamoorthy Ketamine Does Not Increase Pulmonary Vascular Resistance in Children with Pulmonary Hypertension Undergoing Sevoflurane Anesthesia and Spontaneous Ventilation Anesth. Analg., December 1, 2007; 105(6): 1578 - 1584. [Abstract] [Full Text] [PDF]

				P. Bartsch and J. S. R. Gibbs Effect of Altitude on the Heart and the Lungs Circulation, November 6, 2007; 116(19): 2191 - 2202. [Full Text] [PDF]

				M. R. Fisher, G. J. Criner, A. P. Fishman, P. M. Hassoun, O. A. Minai, S. M. Scharf, a. H. E. Fessler, and for the National Emphysema Treatment Trial (NETT) Estimating pulmonary artery pressures by echocardiography in patients with emphysema Eur. Respir. J., November 1, 2007; 30(5): 914 - 921. [Abstract] [Full Text] [PDF]

				F. Reichenberger, R. Voswinckel, B. Enke, M. Rutsch, E. El Fechtali, T. Schmehl, H. Olschewski, R. Schermuly, N. Weissmann, H. A. Ghofrani, et al. Long-term treatment with sildenafil in chronic thromboembolic pulmonary hypertension Eur. Respir. J., November 1, 2007; 30(5): 922 - 927. [Abstract] [Full Text] [PDF]

				M. A. Aldred, R. D. Machado, V. James, N. W. Morrell, and R. C. Trembath Characterization of the BMPR2 5'-Untranslated Region and a Novel Mutation in Pulmonary Hypertension Am. J. Respir. Crit. Care Med., October 15, 2007; 176(8): 819 - 824. [Abstract] [Full Text] [PDF]