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      Should we use the oral selective IP receptor agonist selexipag off-label in children with pulmonary arterial hypertension?

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          Abstract

          We discuss the currently available data on the use of the prostacyclin mimetic selexipag in children and adolescents with pulmonary arterial hypertension (PAH). Future indications may include transitioning from intravenous prostacyclin/prostacyclin analog to oral selexipag, and vice versa, or adding selexipag as a third oral PAH-targeted agent in children not responding well to dual PAH therapy.

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          Selexipag for the Treatment of Pulmonary Arterial Hypertension.

          Olivier Sitbon, Richard W. Channick, Kelly Chin … (2015)
          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.
          Article 0 comments Cited 306 times – based on 0 reviews     Review now
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            Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study.

            Olivier Sitbon, Xavier Jaïs, Laurent Savale … (2014)
            Patients with severe pulmonary arterial hypertension (PAH) in New York Heart Association (NYHA) functional class (FC) III/IV have a poor prognosis, despite survival benefits being demonstrated with intravenous epoprostenol. In this pilot study, the efficacy and safety of a triple combination therapy regimen in patients with severe PAH was investigated. Data from newly diagnosed NYHA FC III/IV PAH patients (n=19) initiated on upfront triple combination therapy (intravenous epoprostenol, bosentan and sildenafil) were collected retrospectively from a prospective registry. Significant improvements in 6-min walk distance and haemodynamics were observed after 4 months' triple combination therapy in 18 patients (p<0.01); 17 patients had improved to NYHA FC I or II. One patient was not included in the month 4 assessment (due to an emergency lung transplant in month 3). At the final evaluation (mean ± sd 32 ± 19 months), all 18 patients had sustained clinical and haemodynamic improvement. Overall survival estimates for the triple combination cohort were 100% at 1, 2 and 3 years. Expected survival calculated from the French equation was 75% (95% CI 68-82%), 60% (95% CI 50-70%) and 49% (95% CI 38-60%) at 1, 2 and 3 years, respectively. This pilot study provides preliminary evidence of the long-term benefits of upfront triple combination therapy in patients with severe PAH.
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              Pulmonary Hypertension in Infants, Children, and Young Adults.

              Georg Hansmann (2017)
              Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure. Recent advancements in PH-targeted therapies and interventional-surgical procedures have contributed to the improvement in quality of life and survival in PH/PHVD. This paper gives an update on recent developments in the diagnosis and treatment of children and young adults with PH. The focus is on the heterogeneous etiology/pathophysiology of PH in the young, and particularly on PHVD associated with congenital heart disease. Moreover, new pharmacological, surgical, and interventional therapies and their practical application in progressive/severe pulmonary arterial hypertension with inadequate response to conventional pharmacotherapy are discussed.
              Article 0 comments Cited 55 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Pulm Circ
                Journal ID (iso-abbrev): Pulm Circ
                Journal ID (publisher-id): PUL
                Journal ID (hwp): sppul
                Title: Pulmonary Circulation
                Publisher: SAGE Publications (Sage UK: London, England )
                ISSN (Print): 2045-8932
                ISSN (Electronic): 2045-8940
                Publication date (Electronic): 27 September 2018
                Publication date Collection: Jul-Sep 2018
                Volume: 8
                Issue: 3
                Electronic Location Identifier: 2045894018793580
                Affiliations
                [1 ]Division of Pediatric Cardiology, Department of Pediatrics, Medical University of Graz, Austria
                [2 ]Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Germany
                Author notes
                [*]Georg Hansmann, Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Carl-Neuberg-Str. 1, Hannover 30625, Germany. Email: georg.hansmann@ 123456gmail.com Martin Koestenberger, Department of Pediatrics, Medical University of Graz, Auenbruggerplatz 34/2, Graz 8036, Austria. Email: martin.koestenberger@ 123456medunigraz.at
                Article
                Publisher ID: 10.1177_2045894018793580
                DOI: 10.1177/2045894018793580
                PMC ID: 6161208
                PubMed ID: 30260767
                SO-VID: 3633805f-09a8-4217-aa35-df5835fe5fe2
                Copyright © © The Author(s) 2018
                License:

                Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.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
                Date received : 27 April 2018
                Date accepted : 14 July 2018
                Funding
                Funded by: Deutsche Forschungsgemeinschaft, FundRef https://doi.org/10.13039/501100001659;
                Award ID: German Research Foundation (DFG; HA 4348/6-1)
                Categories
                Subject: Research Letter
                Custom metadata
                cover-date July-September 2018

                ScienceOpen disciplines: Respiratory medicine
                Keywords: pediatric,prostacyclin receptor agonist,pulmonary arterial hypertension,selexipag
                Data availability:
                ScienceOpen disciplines: Respiratory medicine
                Keywords: pediatric, prostacyclin receptor agonist, pulmonary arterial hypertension, selexipag

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