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      Real-world experience using combination therapy with riociguat and risk assessment using REVEAL Lite 2.0 in patients with pulmonary arterial hypertension

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          Abstract

          Pulmonary arterial hypertension is a progressive disease that can lead to right-sided ventricular failure and premature death. Tailoring therapy to individual patient’s needs, along with regular risk assessment, is integral for optimal outcomes in patients with pulmonary arterial hypertension. Results from the AMBITION trial support the use of upfront combination of tadalafil and ambrisentan. In a recent analysis of risk assessment in pulmonary arterial hypertension, abridged versions of the REVEAL 2.0 risk score were shown to be comparable to the full tools. In this report, we present a case series of the use of riociguat in upfront combination or sequentially, and the impact on risk scores as determined by the abridged REVEAL Lite 2.0 approach.

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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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            Combination therapy in pulmonary arterial hypertension: recent accomplishments and future challenges

            Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by a progressive increase in pulmonary vascular resistance, ultimately leading to right heart failure and death. Throughout the past 20 years, numerous specific pharmacologic agents, including phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostaglandins, and more recently, soluble guanylate cyclase stimulators and selective IP prostacyclin receptor agonist, have emerged for the treatment of PAH. Early clinical trials were typically of short-term duration, comparing the effects of PAH-targeted therapies versus placebo and using exercise tolerance as the primary endpoint in most trials. A meta-analysis of these trials documented a reduction in short-term mortality of ∼40% with monotherapy. More recently, we have witnessed a progressive shift in PAH study designs using longer event-driven trials comparing the effects of upfront and sequential combination therapy on clinical worsening that is perceived as a more clinically relevant outcome measure. Recent meta-analyses also documented that combination therapy significantly reduced the risk of clinical worsening by ∼35% compared with monotherapy alone. In this review article, we will discuss the evolution of treatments and clinical trial design in the field of PAH over the past decades with a special focus on combination therapy and its current role in the management of PAH. We will also detail unresolved questions regarding the future of PAH patients’ care and the challenges of future clinical trials.
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              Therapy for pulmonary arterial hypertension in adults 2018: update of the chest guideline and expert panel report

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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
                28 February 2020
                Jan-Mar 2020
                : 10
                : 1
                : 2045894020910098
                Affiliations
                [1-2045894020910098]Houston Methodist Hospital, Houston, USA
                Author notes
                [*]Sandeep Sahay, Institute of Academic Faculty, Weill Cornell Medicine, Houston Methodist Lung Center, Houston Methodist Hospital, Houston, TX 77030, USA. Email: ssahay@ 123456houstonmethodist.org
                Article
                10.1177_2045894020910098
                10.1177/2045894020910098
                7052464
                9291ef64-165f-48f1-b1b7-89eda06e31cf
                © 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
                : 19 November 2019
                : 12 December 2019
                Categories
                Case Series
                Custom metadata
                January-March 2020
                ts2

                Respiratory medicine
                chronic thromboembolic pulmonary hypertension,pulmonary arterial hypertension,pulmonary hypertension,riociguat

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