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      Pulmonary Arterial Hypertension: Diagnosis, Treatment, and Novel Advances

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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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            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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              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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                Author and article information

                Contributors
                (View ORCID Profile)
                (View ORCID Profile)
                Journal
                American Journal of Respiratory and Critical Care Medicine
                Am J Respir Crit Care Med
                American Thoracic Society
                1073-449X
                1535-4970
                June 15 2021
                June 15 2021
                : 203
                : 12
                : 1472-1487
                Affiliations
                [1 ]Division of Cardiovascular Medicine, Brigham and Women’s Hospital and Harvard Medical School, Harvard University, Boston, Massachusetts;
                [2 ]Section of Pulmonary Medicine, Children’s Hospital Colorado and the University of Colorado Anschutz Medical Center, University of Colorado, Aurora, Colorado;
                [3 ]Intermountain Medical Center and the University of Utah, Salt Lake City, Utah;
                [4 ]Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota;
                [5 ]Division of Pediatric Cardiology, Department of Pediatrics, School of Medicine, Stanford University, Palo Alto, California;
                [6 ]Division of Cardiology, Department of Medicine, Weill Cornell Graduate School of Medical Sciences, Cornell University, New York, New York;
                [7 ]Veterans Affairs Palo Alto Health Care System and School of Medicine, Stanford University, Stanford, California;
                [8 ]Advanced Lung Disease and Transplant Program, Inova Fairfax Hospital, Falls Church, Virginia;
                [9 ]Division of Cardiology, Department of Medicine, Feinberg School of Medicine, Northwestern University, Chicago, Illinois;
                [10 ]Department of Pulmonology, Medical University of Graz and Ludwig Boltzmann Institute for Lung Vascular Research, Graz, Austria; and
                [11 ]Department of Pediatrics and
                [12 ]Department of Medicine, Columbia University Vagelos College of Physicians and Surgeons, New York, New York
                Article
                10.1164/rccm.202012-4317SO
                33861689
                deb706a0-f06b-4472-9fab-54b46408f2e5
                © 2021
                History

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