Updated Clinical Classification of Pulmonary Hypertension

Simonneau, Gérald; Robbins, Ivan M.; Beghetti, Maurice; Channick, Richard N.; Delcroix, Marion; Denton, Christopher P.; Elliott, C. Gregory; Gaine, Sean P.; Gladwin, Mark T.; Jing, Zhi-Cheng; Krowka, Michael J.; Langleben, David; Nakanishi, Norifumi; Souza, Rogério

doi:10.1016/j.jacc.2009.04.012

« Previous Next »Journal of the American College of Cardiology
Volume 54, Issue 1, Supplement , Pages S43-S54, 30 June 2009

Updated Clinical Classification of Pulmonary Hypertension

Gérald Simonneau, MD
- Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud Hôpital Antoine Béclère, Clamart, France
- 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
Ivan M. Robbins, MD
- Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee
Maurice Beghetti, MD
- Pediatric Cardiology Unit, Hôpital des Enfants, University Hospital of Geneva, Geneva, Switzerland
Richard N. Channick, MD
- Division of Pulmonary and Critical Care Medicine, UCSD Medical Center, La Jolla, California
Marion Delcroix, MD, PhD
- Center for Pulmonary Vascular Disease, Department of Pneumology, Gasthuisberg University Hospital, Leuven, Belgium
Christopher P. Denton, MD, PhD
- Centre for Rheumatology, Royal Free Hospital, London, United Kingdom
C. Gregory Elliott, MD
- Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, Utah
Sean P. Gaine, MD, PhD
- Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland
Mark T. Gladwin, MD
- Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, Pennsylvania
Zhi-Cheng Jing, MD
- Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China
Michael J. Krowka, MD
- Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota
David Langleben, MD
- Center for Pulmonary Vascular Disease, Sir Mortimer B. Davis Jewish General Hospital, Montréal, Québec, Canada
Norifumi Nakanishi, MD, PhD
- Division of Cardiology and Pulmonary Circulation, Department of Internal Medicine National Cardiovascular Center, Osaka, Japan
Rogério Souza, MD
- Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil

Received 6 February 2009; accepted 15 April 2009.

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