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      The 6th World Symposium on Pulmonary Hypertension: what’s old is new

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          Abstract

          In February 2018, the 6th World Symposium on Pulmonary Hypertension (WSPH) brought together experts from various disciplines to review the most relevant clinical and scientific advances in the field of PH over the last 5 years. Based on careful review and discussions by members of the different task forces, major revisions were made on the hemodynamic definition for various forms of PH and new genes were added to the list of genetic markers associated with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease. In addition, the use of risk stratification tools was encouraged as a strategy to reduce one-year mortality risk in PAH patients through early implementation of PAH therapies. While members of the medical community are still debating some of the proposed changes, the new WSPH guidelines advocate early diagnosis and initiation of combination therapy to reduce mortality and improve quality of life in patients with PH.

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          Most cited references24

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          Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension.

          Data on the effect of initial combination therapy with ambrisentan and tadalafil on long-term outcomes in patients with pulmonary arterial hypertension are scarce.
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            Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review.

            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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              Five-Year outcomes of patients enrolled in the REVEAL Registry.

              Pulmonary arterial hypertension (PAH) is a rare, severe disease characterized by worsening right-sided heart failure, decreasing functional status, and poor survival. The present study characterizes the 5-year survival in the United States of a new and previous diagnosis of PAH in patients stratified by baseline functional class (FC). The Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) is a 55-center observational US registry of the demographics, disease course, and management of patients with World Health Organization (WHO) group 1 PAH.
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                Author and article information

                Contributors
                Role: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Role: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Role: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Role: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Role: ConceptualizationRole: Funding AcquisitionRole: SupervisionRole: Writing – Original Draft PreparationRole: Writing – Review & Editing
                Journal
                F1000Res
                F1000Res
                F1000Research
                F1000Research
                F1000 Research Limited (London, UK )
                2046-1402
                19 June 2019
                2019
                : 8
                : F1000 Faculty Rev-888
                Affiliations
                [1 ]Division of Pulmonary and Critical Care Medicine, Stanford University, Stanford, USA
                [2 ]Vera Moulton Wall Center for Pulmonary Vascular Research, Stanford University, Stanford, USA
                Author notes

                No competing interests were disclosed.

                Author information
                https://orcid.org/0000-0001-5532-8247
                Article
                10.12688/f1000research.18811.1
                6584967
                31249672
                666360c9-5f09-4108-916c-9270b17b96d1
                Copyright: © 2019 Condon DF et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 11 June 2019
                Funding
                Funded by: NIH
                Award ID: R01HL134776
                Award ID: R01HL134776-02
                This work was supported by National Institute of Health grants R01 HL134776 and R01 HL139664 (to VAdJP).
                The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
                Categories
                Review
                Articles

                pulmonary hypertension,hemodynamics,therapy,genetics
                pulmonary hypertension, hemodynamics, therapy, genetics

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