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J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D34-41. doi: 10.1016/j.jacc.2013.10.029.

Updated clinical classification of pulmonary hypertension.

Author information

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:
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, United Kingdom.
University of Alberta, Stollery Children's Hospital and Mazankowski Alberta Heart Institute, Edmonton, Alberta, Canada.
Heart Research Institute, Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia.
Centre for Rheumatology and Connective Tissue Diseases, Division of Medicine, Royal Free Campus, UCL Medical School, London, United Kingdom.
University of Giessen and Marburg Lung Center, Geissen, Hesse, Germany.
Cardiology Service, Hospital Universitario 12 de Octubre, Madrid, Spain.
Pediatric Cardiology, Amrita Institute of Medical Sciences, Cochin, Kerala, India.
Childrens' Hospital, Boston, Massachusetts.
University of Illinois, Chicago, Illinois.
Institute for Lung and Vascular Research, Medical University of Graz, Graz, Austria.
Vanderbilt University Medical Center, Nashville, Tennessee.
Pulmonary Department, Heart Institute, University of São Paulo, Medical School, São Paulo, Brazil.

Erratum in

  • J Am Coll Cardiol. 2014 Feb 25;63(7):746.
  • Correction. [J Am Coll Cardiol. 2014]


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.


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

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