title: Updated clinical classification of pulmonary hypertension.
abstract: In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.
authors.affiliation: Assistance publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Universitaire de Bicêtre, Université Paris-Sud, Laboratoire d'excellence en recherche sur le médicament et innovation thérapeutique, and INSERM, Unité 999, Le Kremlin Bicêtre, France. Electronic address: gerald.simonneau@bct.aphp.fr.
authors.forename: Gerald
authors.initials: G
authors.lastname: Simonneau
authors.affiliation: Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, United Kingdom.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: University of Alberta, Stollery Children's Hospital and Mazankowski Alberta Heart Institute, Edmonton, Alberta, Canada.
authors.forename: Ian
authors.initials: I
authors.lastname: Adatia
authors.affiliation: Heart Research Institute, Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia.
authors.forename: David
authors.initials: D
authors.lastname: Celermajer
authors.affiliation: Centre for Rheumatology and Connective Tissue Diseases, Division of Medicine, Royal Free Campus, UCL Medical School, London, United Kingdom.
authors.forename: Chris
authors.initials: C
authors.lastname: Denton
authors.affiliation: University of Giessen and Marburg Lung Center, Geissen, Hesse, Germany.
authors.forename: Ardeschir
authors.initials: A
authors.lastname: Ghofrani
authors.affiliation: Cardiology Service, Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Miguel Angel
authors.initials: MA
authors.lastname: Gomez Sanchez
authors.affiliation: Pediatric Cardiology, Amrita Institute of Medical Sciences, Cochin, Kerala, India.
authors.forename: R
authors.initials: R
authors.lastname: Krishna Kumar
authors.affiliation: Childrens' Hospital, Boston, Massachusetts.
authors.forename: Michael
authors.initials: M
authors.lastname: Landzberg
authors.affiliation: University of Illinois, Chicago, Illinois.
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.affiliation: Institute for Lung and Vascular Research, Medical University of Graz, Graz, Austria.
authors.forename: Horst
authors.initials: H
authors.lastname: Olschewski
authors.affiliation: Vanderbilt University Medical Center, Nashville, Tennessee.
authors.forename: Ivan M
authors.initials: IM
authors.lastname: Robbins
authors.affiliation: Pulmonary Department, Heart Institute, University of São Paulo, Medical School, São Paulo, Brazil.
authors.forename: Rogiero
authors.initials: R
authors.lastname: Souza
mesh_terms: D000743:Anemia, Hemolytic / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology; D000818:Animals; D003240:Connective Tissue Diseases / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification* / Q000175:diagnosis* / Q000453:epidemiology
keywords: CHD; HAART; HIV; IFN; PAH; PAP; PH; PH due to chronic lung diseases; PH due to left heart disease; POPH; PPHN; PVR; SCD; Sch-PAH; TGF; TKI; chronic thromboembolic pulmonary hypertension; congenital heart disease; highly active antiretroviral therapy; human immunodeficiency virus; interferon; persistent pulmonary hypertension of the newborn; portopulmonary hypertension; pulmonary arterial hypertension; pulmonary arterial pressure; pulmonary hypertension; pulmonary vascular resistance; schistosomiasis-associated PAH; sickle cell disease; tumor growth factor; tyrosine kinase inhibitor