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Archives of the Turkish Society of Cardiology
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Updated clinical classification of pulmonary hypertension [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2014; 42(1): 45-54

Updated clinical classification of pulmonary hypertension

Gerald Simonneau1, Michael A. Gatzoulis2, Ian Adatia3, David Celermajer4, Chris Denton5, Ardeschir Ghofrani6, Miguel Angel Gomez Sanchez7, R. Krishna Kumar8, Michael Landzberg9, Roberto F. Machado10, Horst Olschewski11, Ivan M. Robbins12, Rogiero Souza13
1Paris Public Hospital, Pneumology Service, Bicêtre University Hospital, University of Paris-Sud, "Laboratoire d'Excellence en Recherche et Innovation Therapeutique sur le medicament, and INSERM, Unité 999", Le Kremlin Bicetre, France
2Imperial College, Royal Brompton Hospital and National Heart and Lung Institute, Adult Congenital Heart and Pulmonary Hypertension Centre, London, England
3University of Alberta, Stollery Children's Hospital and Mankowski Alberta Heart Institute in Edmonton, Alberta, Canada
4The University of Sydney, Royal Prince Alfred Hospital, Heart Research Institute, Sydney, Australia
5UCL School of Medicine, "the Royal Free Campus", Rheumatology and Connective Tissue Disorders Center, Department of Medicine, London, England
6University of Giessen and Marburg Lung Center, Giessen, Hesse, Germany
7October 12 University Hospital, Department of Cardiology, Madrid, Spain
8Amrita Institute of Medical Sciences, Department of Pediatric Cardiology, Cochin, Kerala, India
9Children's Hospital, Boston, Massachusetts, USA
10University of Illinois at Chicago, Illinois, USA
11Graz School of Medicine, Pulmonary and Vascular Research Institute, Graz, Austria
12Vanderbilt University Medical Center, Nashville, Tennessee, USA
13São Paulo University School of Medicine, Pulmonary Division, Heart Institute, São Paulo, Brazil

Mn 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 (YPPH) 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. (J Am Coll Cardiol 2013;62: D34–41) ª 2013 by the American College of Cardiology Foundation.

Keywords: Khronic thromboembolic pulmonary hypertension, PH due to chronic lung diseases, PH due to left heart disease, pulmonary arterial hypertension; pulmonary hypertension

How to cite this article
Gerald Simonneau, Michael A. Gatzoulis, Ian Adatia, David Celermajer, Chris Denton, Ardeschir Ghofrani, Miguel Angel Gomez Sanchez, R. Krishna Kumar, Michael Landzberg, Roberto F. Machado, Horst Olschewski, Ivan M. Robbins, Rogiero Souza. Updated clinical classification of pulmonary hypertension. Turk Kardiyol Dern Ars. 2014; 42(1): 45-54

Corresponding Author: Gerald Simonneau, France
Manuscript Language: Turkish


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