title: Updated clinical classification of pulmonary hypertension.
abstract: 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.
authors: Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud Hôpital Antoine Béclère, Clamart, France. Electronic address: gerald.simonneau@abc.aphp.fr.
authors: Gérald
authors: G
authors: Simonneau
authors: Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
authors: Ivan M
authors: IM
authors: Robbins
authors: Pediatric Cardiology Unit, Hôpital des Enfants, University Hospital of Geneva, Geneva, Switzerland.
authors: Maurice
authors: M
authors: Beghetti
authors: Division of Pulmonary and Critical Care Medicine, UCSD Medical Center, La Jolla, California.
authors: Richard N
authors: RN
authors: Channick
authors: Center for Pulmonary Vascular Disease, Department of Pneumology, Gasthuisberg University Hospital, Leuven, Belgium.
authors: Marion
authors: M
authors: Delcroix
authors: Centre for Rheumatology, Royal Free Hospital, London, United Kingdom.
authors: Christopher P
authors: CP
authors: Denton
authors: Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, Utah.
authors: C Gregory
authors: CG
authors: Elliott
authors: Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland.
authors: Sean P
authors: SP
authors: Gaine
authors: Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, Pennsylvania.
authors: Mark T
authors: MT
authors: Gladwin
authors: Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China.
authors: Zhi-Cheng
authors: ZC
authors: Jing
authors: Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota.
authors: Michael J
authors: MJ
authors: Krowka
authors: Center for Pulmonary Vascular Disease, Sir Mortimer B. Davis Jewish General Hospital, Montréal, Québec, Canada.
authors: David
authors: D
authors: Langleben
authors: Division of Cardiology and Pulmonary Circulation, Department of Internal Medicine National Cardiovascular Center, Osaka, Japan.
authors: Norifumi
authors: N
authors: Nakanishi
authors: Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
authors: Rogério
authors: R
authors: Souza
mesh_terms: D000465:Algorithms; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000008:administration & dosage; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D015897:Comorbidity; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol / Q000008:administration & dosage; D019317:Evidence-Based Medicine; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000188:drug therapy* / Q000453:epidemiology; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D012595:Scleroderma, Systemic / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol