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      Transition from selexipag to oral treprostinil in a patient with pulmonary arterial hypertension

      case-report

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          Abstract

          Prostacyclins are the mainstay treatment for patients with severe pulmonary arterial hypertension. This case highlights the transition from selexipag to oral treprostinil. Our patient improved both subjectively and objectively. Cardiac output and index, as measured by the echocardiogram, improved 12% and 7.7%, respectively. Invasive hemodynamic data revealed greater improvements: cardiac output improved by 25% and cardiac index by 28%. Mixed venous oxygen saturation improved from 65% to 71%. A possible explanation is that selexipag has a maximal dose, whereas there is no recommended maximum dose of oral treprostinil. Another theory is oral treprostinil has higher affinity to the IP receptor, though selexipag has a higher specificity. However, there are no bio-equivalency data, and data comparing pharmacodynamics of both drugs are lacking. Furthermore, no head-to-head trials comparing these agents exist.

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          Most cited references6

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          Selexipag for the Treatment of Pulmonary Arterial Hypertension.

          In a phase 2 trial, selexipag, an oral selective IP prostacyclin-receptor agonist, was shown to be beneficial in the treatment of pulmonary arterial hypertension.
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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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              Oral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trial.

              Infused and inhaled treprostinil are effective for treatment of pulmonary arterial hypertension (PAH), although their administration routes have limitations. This study assessed the efficacy and safety of bid oral sustained-release treprostinil in the treatment of PAH with a concomitant endothelin receptor antagonist (ERA) and/or phosphodiesterase type 5 inhibitor.
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                Author and article information

                Journal
                Pulm Circ
                Pulm Circ
                PUL
                sppul
                Pulmonary Circulation
                SAGE Publications (Sage UK: London, England )
                2045-8932
                2045-8940
                8 April 2020
                Jan-Mar 2020
                : 10
                : 1
                : 2045894019898032
                Affiliations
                [1 ]Department of Internal Medicine, Houston Methodist Hospital, Weill Cornell College, Houston, USA
                [2 ]Houston Methodist Lung Center, Houston Methodist Hospital, Weill Cornell College, Houston, USA
                Author notes
                [*]Ifeoma Oriaku, Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, 3401 N Broad St., Philadelphia, PA, USA. Email: ioriaku23@ 123456gmail.com
                Author information
                https://orcid.org/0000-0002-8984-8205
                Article
                10.1177_2045894019898032
                10.1177/2045894019898032
                7144673
                360397cc-db12-4c49-b90c-9456abdd5116
                © The Author(s) 2020

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 1 June 2019
                : 23 November 2019
                Categories
                Case Report
                Custom metadata
                January-March 2020
                ts2

                Respiratory medicine
                pulmonary arterial hypertension,echocardiography,hemodynamics,angiography,prostaglandins

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