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      Pulmonary hypertension and chronic lung disease: where are we headed?

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          Abstract

          Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide. Therefore, it is desirable to develop useful and reliable tools to obtain an early diagnosis and to monitor and follow-up this condition, while new insights in the therapeutic approach are explored.

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          New prospective in the management of pulmonary hypertension related to chronic lung diseases http://bit.ly/2lW4CfE

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          Most cited references130

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          ATS statement: guidelines for the six-minute walk test.

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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).

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              Haemodynamic definitions and updated clinical classification of pulmonary hypertension

              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.
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                Author and article information

                Journal
                Eur Respir Rev
                Eur Respir Rev
                ERR
                errev
                European Respiratory Review
                European Respiratory Society
                0905-9180
                1600-0617
                30 September 2019
                18 October 2019
                : 28
                : 153
                : 190065
                Affiliations
                [1 ]U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
                [2 ]U.O. di Radiologia Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
                [3 ]Radiologia, Università di Bologna. Ospedale O. Malpighi, Bologna, Italy
                [4 ]U.O. di Medicina Generale, Ospedale San Giuseppe - MultiMedica IRCCS, Milan, Italy
                [5 ]Servizio di Anatomia Patologica, Polo Scientifico e Tecnologico, Multimedica Srl, IRCCS, Milan, Italy
                [6 ]Institut de Cardiologie et de Pneumologie de Québec, Quebec, Canada
                Author notes
                Antonella Caminati, U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe - MultiMedica IRCCS, via San Vittore 12, Milan 20123, Italy. E-mail lafitta@ 123456libero.it
                Author information
                https://orcid.org/0000-0001-8629-7391
                Article
                ERR-0065-2019
                10.1183/16000617.0065-2019
                9488824
                31636088
                cb28dc03-186a-4066-955d-91d19e1e12f2
                Copyright ©ERS 2019.

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

                History
                : 09 June 2019
                : 22 September 2019
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