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      Haemodynamic definitions and updated clinical classification of pulmonary hypertension


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          Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management.

          Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”, due to the specific prognostic and management of these patients, and a subgroup “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement”, due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.


          State of the art and research perspectives of haemodynamic definitions and clinical classification of pulmonary hypertension http://ow.ly/TJeR30mgWKj

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          Most cited references86

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          2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).

          Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk-benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.
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            Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry.

            The Registry to EValuate Early And Long-term pulmonary arterial hypertension disease management (REVEAL Registry) was established to provide updated characteristics of patients with pulmonary arterial hypertension (PAH) and to improve diagnosis, treatment, and management. Fifty-four US centers enrolled consecutively screened patients with World Health Organization group I PAH who met expanded hemodynamic criteria of mean pulmonary arterial pressure (PAP) > 25 mm Hg at rest (30 mm Hg with exercise), pulmonary capillary wedge pressure (PCWP) or= 240 dynes x s x cm(-5). Patients meeting the traditional hemodynamic definition (PCWP
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              Updated clinical classification of pulmonary hypertension.

              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. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

                Author and article information

                Eur Respir J
                Eur. Respir. J
                The European Respiratory Journal
                European Respiratory Society
                January 2019
                24 January 2019
                : 53
                : 1
                : 1801913
                [1 ]Université Paris-Sud, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Service de Pneumologie, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Hôpital de Bicêtre, Le Kremlin-Bicêtre, France
                [2 ]INSERM UMR_S999, LabEx LERMIT, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
                [3 ]Faculty of Medicine and Health, The University of Sydney, Sydney, Australia
                [4 ]Centre for Rheumatology, Royal Free Campus, University College London, London, UK
                [5 ]Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Trust, and the National Heart and Lung Institute, Imperial College London, London, UK
                [6 ]Transplant Center, Mayo Clinic, Rochester, MN, USA
                [7 ]Center of Chest Disease and Critical Care, Milpark Hospital, Johannesburg, South Africa
                [8 ]Pulmonary Circulation Unit, Pulmonary Division, Heart Institute (InCor), Hospital das Clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil
                Author notes
                Gérald Simonneau, Service de Pneumologie, Centre de Référence de l'Hypertension Pulmonaire, CHU de Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre Cedex, France. E-mail: gerald.simonneau@ 123456aphp.fr
                Author information
                Copyright ©ERS 2019

                This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.

                : 06 October 2018
                : 09 October 2018
                World Symposium on Pulmonary Hypertension

                Respiratory medicine
                Respiratory medicine


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