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STATE-OF-THE-ART PAPER

Updated Clinical Classification of Pulmonary Hypertension

Gérald Simonneau, MD^_*,_*, Ivan M. Robbins, MD, Maurice Beghetti, MD, Richard N. Channick, MD, Marion Delcroix, MD, PhD^||, Christopher P. Denton, MD, PhD^¶, C. Gregory Elliott, MD^#, Sean P. Gaine, MD, PhD^_**, Mark T. Gladwin, MD, Zhi-Cheng Jing, MD, Michael J. Krowka, MD, David Langleben, MD^||||, Norifumi Nakanishi, MD, PhD^¶¶ and Rogério Souza, MD^##

^_* Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud Hôpital Antoine Béclère, Clamart, France
Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
Pediatric Cardiology Unit, Hôpital des Enfants, University Hospital of Geneva, Geneva, Switzerland
Division of Pulmonary and Critical Care Medicine, UCSD Medical Center, La Jolla, California
^|| Center for Pulmonary Vascular Disease, Department of Pneumology, Gasthuisberg University Hospital, Leuven, Belgium
^¶ Centre for Rheumatology, Royal Free Hospital, London, United Kingdom
^# Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, Utah
^_** Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland
Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, Pennsylvania
Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
^|||| Center for Pulmonary Vascular Disease, Sir Mortimer B. Davis Jewish General Hospital, Montréal, Québec, Canada
^¶¶ Division of Cardiology and Pulmonary Circulation, Department of Internal Medicine National Cardiovascular Center, Osaka, Japan
^## Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil

Manuscript received February 6, 2009; accepted April 15, 2009.

^_* Reprint requests and correspondence: Dr. Gérald Simonneau, Centre National de Référence des Maladies Vasculaires Pulmonaires, Service de Pneumologie, Hôpital Antoine Béclère, Assistance Publique, Hôpitaux de Paris, Université Paris-Sud (XI), 157, rue de la Porte de Trivaux, 92140 Clamart, France (Email: gerald.simonneau{at}abc.aphp.fr).

The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications. The modifications adopted during this meeting principally concern Group 1, pulmonary arterial hypertension (PAH). This subgroup includes patients with PAH with a family history or patients with idiopathic PAH with germline mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the new classification, schistosomiasis and chronic hemolytic anemia appear as separate entities in the subgroup of PAH associated with identified diseases. Finally, it was decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in a separate group, distinct from but very close to Group 1 (now called Group 1'). Thus, Group 1 of PAH is now more homogeneous.

Key Words: pulmonary hypertension • clinical classification • pulmonary arterial hypertension

Abbreviations and Acronyms   BMPR2 = bone morphogenetic protein receptor type 2   CHD = congenital heart disease   CTEPH = chronic thromboembolic pulmonary hypertension   ESRD = end-stage renal disease   HIV = human immunodeficiency virus   IPAH = idiopathic pulmonary arterial hypertension   OR = odds ratio   PAH = pulmonary arterial hypertension   PAP = pulmonary arterial pressure   PCH = pulmonary capillary hemangiomatosis   PH = pulmonary hypertension   POPH = portopulmonary hypertension   PPH = primary pulmonary hypertension   PVOD = pulmonary veno-occlusive disease   PVR = pulmonary vascular resistance   SCD = sickle cell disease   TRV = tricuspid regurgitation jet velocity

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