Updated clinical classification of pulmonary hypertension.
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.
Simonneau
Gérald
G
Centre National de Référence des Maladies Vasculaires Pulmonaires, Université Paris-Sud Hôpital Antoine Béclère, Clamart, France. Electronic address: gerald.simonneau@abc.aphp.fr.
Robbins
Ivan M
IM
Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Beghetti
Maurice
M
Pediatric Cardiology Unit, Hôpital des Enfants, University Hospital of Geneva, Geneva, Switzerland.
Channick
Richard N
RN
Division of Pulmonary and Critical Care Medicine, UCSD Medical Center, La Jolla, California.
Delcroix
Marion
M
Center for Pulmonary Vascular Disease, Department of Pneumology, Gasthuisberg University Hospital, Leuven, Belgium.
Denton
Christopher P
CP
Centre for Rheumatology, Royal Free Hospital, London, United Kingdom.
Elliott
C Gregory
CG
Department of Medicine, Intermountain Medical Center, University of Utah, Salt Lake City, Utah.
Gaine
Sean P
SP
Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland.
Gladwin
Mark T
MT
Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh, Pittsburgh, Pennsylvania.
Jing
Zhi-Cheng
ZC
Department of Pulmonary Circulation, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China.
Krowka
Michael J
MJ
Department of Pulmonary and Critical Care Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota.
Langleben
David
D
Center for Pulmonary Vascular Disease, Sir Mortimer B. Davis Jewish General Hospital, Montréal, Québec, Canada.
Nakanishi
Norifumi
N
Division of Cardiology and Pulmonary Circulation, Department of Internal Medicine National Cardiovascular Center, Osaka, Japan.
Souza
Rogério
R
Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
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
D000925:Anticoagulants; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D011464:Epoprostenol