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      An overview of the 6th World Symposium on Pulmonary Hypertension

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          Abstract

          Since 1973 the World Symposia on Pulmonary Hypertension (WSPH) proceedings have summarised the scientific advances and future needs in this field through the efforts of multiple task forces, each focusing on a different aspect of pulmonary hypertension (PH) [1]. The 6th WSPH comprised 124 experts, divided into 13 task forces, that began their work in January 2017 and presented their consensus opinions to an audience of 1376 participant attendees between February 27 and March 1, 2018 in Nice, France. A newly created task force dedicated to patients' perspectives, including representatives of patients' associations worldwide, was added for the 6th WSPH.

          Abstract

          State of the art summary on diagnosis, prognosis, therapy and future perspectives of pulmonary hypertension http://ow.ly/8MHN30mGtqs

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          Pulmonary hypertension in chronic lung disease and hypoxia

          Steven D. Nathan, Joan Barberà, Sean P Gaine … (2019)
          Pulmonary hypertension (PH) frequently complicates the course of patients with various forms of chronic lung disease (CLD). CLD-associated PH (CLD-PH) is invariably associated with reduced functional ability, impaired quality of life, greater oxygen requirements and an increased risk of mortality. The aetiology of CLD-PH is complex and multifactorial, with differences in the pathogenic sequelae between the diverse forms of CLD. Haemodynamic evaluation of PH severity should be contextualised within the extent of the underlying lung disease, which is best gauged through a combination of physiological and imaging assessment. Who, when, if and how to screen for PH will be addressed in this article, as will the current state of knowledge with regard to the role of treatment with pulmonary vasoactive agents. Although such therapy cannot be endorsed given the current state of findings, future studies in this area are strongly encouraged.
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            Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review.

            G. Kovacs, A Berghold, S Scheidl … (2009)
            According to current guidelines, pulmonary arterial hypertension (PAH) is diagnosed when mean pulmonary arterial pressure (Ppa) exceeds 25 mmHg at rest or 30 mmHg during exercise. Issues that remain unclear are the classification of Ppa values 30 mmHg during exercise is always pathological. We performed a comprehensive literature review and analysed all accessible data obtained by right heart catheter studies from healthy individuals to determine normal Ppa at rest and during exercise. Data on 1,187 individuals from 47 studies in 13 countries were included. Data were stratified for sex, age, geographical origin, body position and exercise level. Ppa at rest was 14.0+/-3.3 mmHg and this value was independent of sex and ethnicity. Resting Ppa was slightly influenced by posture (supine 14.0+/-3.3 mmHg, upright 13.6+/-3.1 mmHg) and age ( or = 50 yrs: 14.7+/-4.0 mmHg). Ppa during exercise was dependent on exercise level and age. During mild exercise, Ppa was 19.4+/-4.8 mmHg in subjects aged or = 50 yrs (p<0.001). In conclusion, while Ppa at rest is virtually independent of age and rarely exceeds 20 mmHg, exercise Ppa is age-related and frequently exceeds 30 mmHg, especially in elderly individuals, which makes it difficult to define normal Ppa values during exercise.
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              Chronic thromboembolic pulmonary hypertension

              Nick H Kim, Marion Delcroix, Xavier Jaïs … (2019)
              Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of pulmonary embolism and a major cause of chronic PH leading to right heart failure and death. Lung ventilation/perfusion scintigraphy is the screening test of choice; a normal scan rules out CTEPH. In the case of an abnormal perfusion scan, a high-quality pulmonary angiogram is necessary to confirm and define the pulmonary vascular involvement and prior to making a treatment decision. PH is confirmed with right heart catheterisation, which is also necessary for treatment determination. In addition to chronic anticoagulation therapy, each patient with CTEPH should receive treatment assessment starting with evaluation for pulmonary endarterectomy, which is the guideline recommended treatment. For technically inoperable cases, PH-targeted medical therapy is recommended (currently riociguat based on the CHEST studies), and balloon pulmonary angioplasty should be considered at a centre experienced with this challenging but potentially effective and complementary intervention.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Eur Respir J
                Journal ID (iso-abbrev): Eur. Respir. J
                Journal ID (publisher-id): ERJ
                Journal ID (hwp): erj
                Title: The European Respiratory Journal
                Publisher: European Respiratory Society
                ISSN (Print): 0903-1936
                ISSN (Electronic): 1399-3003
                Publication date Collection: January 2019
                Publication date (Electronic): 24 January 2019
                Volume: 53
                Issue: 1
                Electronic Location Identifier: 1802148
                Affiliations
                [1 ]Dept of Experimental, Diagnostic and Specialty Medicine-DIMES, Alma Mater Studiorum, University of Bologna, Bologna, Italy
                [2 ]The University of Michigan, Cardiovascular Medicine, Ann Arbor, MI, USA
                [3 ]University of California, San Diego, School of Medicine, La Jolla, CA, USA
                [4 ]Univ. Paris–Sud, AP-HP, Centre de Référence de l'Hypertension Pulmonaire Sévère, Service de Pneumologie, Département Hospitalo-Universitaire (DHU) Thorax Innovation (TORINO), Hôpital de Bicêtre, Le Kremlin Bicêtre, France
                [5 ]INSERM UMR_S999, LabEx LERMIT, Hôpital Marie Lannelongue, Le Plessis Robinson, France
                Author notes
                Nazzareno Galiè, Dept of Experimental, Diagnostic and Specialty Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Via Massarenti 9, 40138 Bologna, Italy. E-mail: nazzareno.galie@ 123456unibo.it
                Article
                Publisher ID: ERJ-02148-2018
                DOI: 10.1183/13993003.02148-2018
                PMC ID: 6351332
                PubMed ID: 30552088
                SO-VID: fe7af2fa-2f58-4615-bb9e-e2b507bfc754
                Copyright © Copyright ©ERS 2019
                License:

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

                History
                Date received : 10 November 2018
                Date accepted : 13 November 2018
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                Subject: Editorials

                ScienceOpen disciplines: Respiratory medicine
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                ScienceOpen disciplines: Respiratory medicine

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