TY - JOUR
T1 - Updated Clinical Classification of Pulmonary Hypertension
AU - Simonneau, Gérald
AU - Robbins, Ivan M.
AU - Beghetti, Maurice
AU - Channick, Richard N.
AU - Delcroix, Marion
AU - Denton, Christopher P.
AU - Elliott, C. Gregory
AU - Gaine, Sean P.
AU - Gladwin, Mark T.
AU - Jing, Zhi Cheng
AU - Krowka, Michael J.
AU - Langleben, David
AU - Nakanishi, Norifumi
AU - Souza, Rogério
N1 - Funding Information:
Prof. Simonneau has received honoraria and research grants from Actelion, Bayer Schering, GlaxoSmithKline, Pfizer, and United Therapeutics. Dr. Robbins has received grant/research support from Actelion, BioMarin, Gilead, Pfizer, and United Therapeutics; and has served on advisory boards, steering committees, and/or speaker's bureaus for Actelion, Gilead, and Lung Rx. Dr. Channick has received consulting and speaker fees and research grants from Actelion, Gilead, Pfizer, and United Therapeutics. Dr. Delcroix has received fees for serving as investigator, speaker, consultant, or steering committee member from Actelion, Aventis Pharmaceuticals, Bayer, Eli Lilly, Encysive, Gilead (Myogen), GlaxoSmithKline, Pfizer, Schering, and United Therapeutics; and research grants from Actelion, Encysive, and GlaxoSmithKline. Dr. Denton has received honoraria and consultancy fees from Actelion, BioVitrum, Encysive, Pfizer, and Genzyme Therapeutics, and research funding from Actelion, Aspreva Pharmaceuticals, Encysive, and Genzyme. Dr. Elliott is employed by Intermountain Healthcare. Intermountain Healthcare, with Dr. Elliott as the inventor, has filed a patent application “method for determining vasoreactivity.” Intermountain Healthcare, with Dr. Elliott as Principal Investigator, has received grant support from Actelion, Pfizer, Encysive, CoTherix, and United Therapeutics. Dr. Elliott serves as a member of the Registry to Evaluate Early and Long Term PAH Disease Management sponsored by Actelion. Dr. Gaine has served on advisory boards for Actelion, GlaxoSmithKline, and Pfizer; received honoraria from Actelion, GlaxoSmithKline, Lung Rx, and Pfizer; and conducted clinical trials supported by Actelion, Gilead, Bayer, and United Therapeutics. Dr. Gladwin has received grant support in the form of a Collaborative Research and Development Agreement between the U.S. Government and INO Therapeutics. He is also listed as a co-inventor on a U.S. Government Patent for the use of nitrite salts for cardiovascular indications. Dr. Jing has received travel support, honoraria for speaking, and consulting fees from Actelion and Bayer Schering. Dr. Nakanishi has received a grant for Cardiovascular Disease and a grant to the Respiratory Failure Research Group from the Ministry of Health, Labour and Welfare of Japan. Dr. Souza has received consulting fees from Actelion; lecture fees from Actelion and Pfizer; and travel support from Gilead (Myogen). Drs. Beghetti, Krowka, and Langleben report no conflicts of interest.
PY - 2009/6/30
Y1 - 2009/6/30
N2 - 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.
AB - 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.
KW - clinical classification
KW - pulmonary arterial hypertension
KW - pulmonary hypertension
UR - http://www.scopus.com/inward/record.url?scp=67649579669&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=67649579669&partnerID=8YFLogxK
U2 - 10.1016/j.jacc.2009.04.012
DO - 10.1016/j.jacc.2009.04.012
M3 - Review article
C2 - 19555858
AN - SCOPUS:67649579669
SN - 0735-1097
VL - 54
SP - S43-S54
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 1 SUPPL. 1
ER -