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Ruxu You, Xinyu Qian, Weijing Tang, Tian Xie, Fang Zeng, Jun Chen, Yu Zhang, Jinyu Liu
Objectives: Although many studies have reported on the cost-effectiveness of bosentan for treating pulmonary arterial hypertension (PAH), a systematic review of economic evaluations of bosentan is currently lacking. Objective evaluation of current pharmacoeconomic evidence can assist decision makers in determining the appropriate place in therapy of a new medication. Methods: Systematic literature searches were conducted in English-language databases (MEDLINE, EMBASE, EconLit databases, and the Cochrane Library) and Chinese-language databases (China National Knowledge Infrastructure, WanFang Data, and Chongqing VIP) to identify studies assessing the cost-effectiveness of bosentan for PAH treatments...
2018: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
Madeleine Themanns, Florian Koban, Christian Bergmayr, Alicja Chrzan, Wolfgang Strohmaier, Johannes Haybaeck, Michael Freissmuth, Eva Zebedin-Brandl
Sinusoidal obstruction syndrome (SOS) is a major complication after hematopoietic stem cell transplantation and belongs to a group of diseases increasingly identified as transplant-related systemic endothelial disease. Administration of defibrotide affords some protection against SOS, but the effect is modest. Hence, there is unmet medical need justifying the preclinical search for alternative approaches. Prostaglandins exert protective actions on endothelial cells of various vascular beds. Here, we explored the therapeutic potential of the prostacyclin analog treprostinil to prevent SOS...
December 7, 2018: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
Nour C Bacha, Marilyne Levy, Coralie L Guerin, Bernard Le Bonniec, Annie Harroche, Isabelle Szezepanski, Jean M Renard, Pascale Gaussem, Dominique Israel-Biet, Chantal M Boulanger, David M Smadja
BACKGROUND: Pulmonary arterial hypertension (PAH) results from pulmonary vascular disease and may eventually lead to right heart failure and death. Vasodilator therapy has greatly improved PAH prognosis. Circulating microvesicles are considered as surrogate markers of endothelial and hematopoietic cell activation. AIM: Thus, our purpose was to determine if MVs are upregulated in pediatric PAH such as reported in adult patients, and to analyze the impact of vasodilator therapies on MV count and function...
November 28, 2018: Pediatric Pulmonology
Hossein-Ardeschir Ghofrani
No abstract text is available yet for this article.
November 23, 2018: Lancet Respiratory Medicine
Roela Sadushi-Kolici, Pavel Jansa, Grzegorz Kopec, Adam Torbicki, Nika Skoro-Sajer, Ioana-Alexandra Campean, Michael Halank, Iveta Simkova, Kristof Karlocai, Regina Steringer-Mascherbauer, Miroslav Samarzija, Barbara Salobir, Walter Klepetko, Jaroslav Lindner, Irene M Lang
BACKGROUND: Treprostinil, a prostacyclin analogue, is effective for the treatment of pulmonary arterial hypertension. However, information is scarce regarding treprostinil for treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to examine the efficacy and safety of subcutaneous treprostinil in this setting. METHODS: In this 24-week, randomised, double-blind controlled trial, we enrolled patients with CTEPH, classified as non-operable, or with persistent or recurrent pulmonary hypertension after pulmonary endarterectomy, in six European expert centres in Austria, Czech Republic, Germany, and Poland...
March 2019: Lancet Respiratory Medicine
Sumei Wang, Miao Yu, Xiangchun Zheng, Shangjuan Dong
Pulmonary arterial hypertension (PAH) can be relieved by pharmacological interventions, especially the targeted drug, which is classified into endothelin receptor antagonist, phosphodiesterase 5 inhibitor, prostaglandin I2 , soluble guanylate cyclase stimulator and selective non-prostanoid prostacyclin receptor agonist. To solve the contradictions existing in reported trials and provide a comprehensive guideline for clinical practice. PubMed, Embase, Cochrane library, and were searched. The basic information about the article, trial, arm, intervention, and the detailed data of outcome, including 6 minutes walking distance (6MWD) change, WHO functional class (FC) improvement, Borg dyspnea score (BDS) change, cardiac index (CI) change, mean pulmonary arterial pressure (mPAP) change, mean right arterial pressure (mRAP) change, pulmonary vascular resistance (PVR) change, clinical worsening, hospitalization, death, severe adverse events (SAEs), and withdrawal were extracted...
November 2018: Drug Delivery
Adaani Frost, Munir Janmohamed, Jason S Fritz, John W McConnell, David Poch, Terry Ann Fortin, Chad E Miller, Kelly M Chin, Micah Fisher, Michael Eggert, Colleen McEvoy, Raymond L Benza, Harrison W Farber, Nick H Kim, Thomas Pfister, Yoko Shiraga, Vallerie McLaughlin
BACKGROUND: A long-term trial showed that the oral prostacyclin (PGl2 ) receptor (IP) agonist, selexipag, delayed disease progression in patients with pulmonary arterial hypertension (PAH). Transition to selexipag in patients treated with more burdensome inhaled therapies that target the prostacyclin pathway may be considered by patients and physicians. The Phase 3b, prospective, open-label TRANSIT-1 (Tolerability and Safety of the Transition From Inhaled Treprostinil to Oral Selexipag in Patients With Pulmonary Arterial Hypertension) study evaluated the safety and tolerability of transition from inhaled treprostinil to oral selexipag...
January 2019: Journal of Heart and Lung Transplantation
Christopher Lambers, Christoph Kornauth, Felicitas Oberndorfer, Panja M Boehm, Michael Tamm, Walter Klepetko, Michael Roth
Treprostinil is applied for pulmonary arterial hypertension (PAH) therapy. However, the mechanism by which the drug achieves its beneficial effects in PAH vessels is not fully understood. This study investigated the effects of treprostinil on PDGF-BB induced remodelling parameters in isolated human pulmonary arterial smooth muscle cells (PASMC) of four PAH patients. The production of TGF-β1, CTGF, collagen type-I and -IV, and of fibronectin were determined by ELISA and PCR. The role of cAMP was determined by ELISA and di-deoxyadenosine treatment...
2018: PloS One
Tengke Wang, Jiakai Lu, Qiang Li, Yao Chen, Qing Ye, Jie Gao, Dong Yang, Liyun Zhao, Jiapeng Huang, Jinglan Zhang
BACKGROUND: Pulmonary hypertension during pregnancy carries high mortality rate. The relatively long-acting, specific pulmonary vasodilator treprostinil has been used to improve survival in these parturients. Slow uptitration is performed in most cases, and rapid titration has not been reported in the postpartum period. METHODS: We retrospectively reviewed 17 pregnant patients with severe pulmonary arterial hypertension who were treated with intravenous treprostinil in our institution between 2014 and 2016...
October 12, 2018: Anesthesia and Analgesia
Batool Alsheikh, Othman Aljohani, Nicole G Coufal
We report a rare aetiology of vocal cord paralysis secondary to undiagnosed severe pulmonary hypertension from a de novo ACVRL1 variant identified by whole-genome sequencing. The patient had a partial response to intravenous treprostinil in addition to inhaled nitric oxide, bosentan, and sildenafil.
December 2018: Cardiology in the Young
Sherif M Fanous, Munir Janmohamed
PURPOSE: Safe transition of patients with pulmonary arterial hypertension (PAH) from parenteral treprostinil to oral selexipag therapy in both inpatient and outpatient settings is described. SUMMARY: There is a paucity of published data on how to safely transition patients to oral therapy in the event of complications and problems during parenteral administration of prostacyclins, which can include bloodstream infections, injection-site pain (with use of subcutaneous treprostinil), infusion pump malfunction, and dosing errors due to incorrect dose preparation...
October 9, 2018: American Journal of Health-system Pharmacy: AJHP
Manuel J Richter, Satenik Harutyunova, Tom Bollmann, Simon Classen, Henning Gall, Felix Gerhardt Md, Friedrich Grimminger, Jan Grimminger, Ekkehard Grünig, Stefan Guth, Michael Halank, Alexander Heine, Marius M Hoeper, Hans Klose, Tobias J Lange, Katrin Meyer, Claus Neurohr, Kai Nickolaus, Karen M Olsson, Christian F Opitz, Stephan Rosenkranz, Hans-Jürgen Seyfarth, Christian Warnke, Christoph Wiedenroth, Hossein A Ghofrani, Ralf Ewert
BACKGROUND: We examined safety and long-term outcomes of intravenous treprostinil administered via the implantable LENUS Pro pump in patients with severe pulmonary hypertension (PH). METHODS: Patients with PH undergoing pump implantation between December 2009 and October 2016 in German referral centers were retrospectively analyzed (end of follow-up: May 2017). The primary objective was to determine long-term safety of the implantable pump. Secondary end points were 3-year survival and prognostic relevance of pre-implantation hemodynamics...
October 2018: Journal of Heart and Lung Transplantation
Sandra Lombardi, Martha Kingman, Maribeth Duncan, Samuel Clark Berngard, Timothy Fernandes
The availability of new medications has improved exercise capacity, enhanced quality of life, and extended time to clinical worsening in patients with pulmonary arterial hypertension (PAH). For many of these medications, careful individualized dose titration is required to maximize therapeutic effectiveness while minimizing side effects. In addition, specific routes of administration, including intravenous (IV), subcutaneous (SC), and inhaled administration may present additional challenges for patients and healthcare providers...
October 2018: Respiratory Medicine
Cher Y Enderby, Charles Burger
Background: Treprostinil is a prostacyclin analogue that directly vasodilates pulmonary and systemic arterial vascular beds. The United States Food and Drug Administration approved inhaled treprostinil in July 2009 for the treatment of group 1 pulmonary arterial hypertension. Inhaled treprostinil avoids issues with continuous infusion prostanoids. This study describes a single institutional experience with inhaled treprostinil. Methods: This was a retrospective review of group 1 pulmonary arterial hypertension patients receiving inhaled treprostinil from July 2009 through September 2015...
September 2018: Therapeutic Advances in Chronic Disease
Travis Maestas, Lillian M Hansen, Rebecca R Vanderpool, Ankit A Desai, Sophia Airhart, Shannon M Knapp, Adam Cohen, Jeremy Feldman, Franz P Rischard
Despite the increasing trends, reports on long-term follow-up are limited on transitioning from parenteral to oral treprostinil therapy in patients with pulmonary arterial hypertension (PAH). We investigated both the effectiveness of parenteral to oral treprostinil transition and the characteristics associated with transition failure over a duration of two years. The study included 37 Group I functional class I and II patients with PAH on combination therapy. Patients were excluded if cardiac index ≤2.2 L/min/m2 , right atrial pressure ≥11 mmHg, or 6-min walk distance ≤250 m...
October 2018: Pulmonary Circulation
Jigisha A Patel, Lei Shen, Susan M Hall, Chabha Benyahia, Xavier Norel, Robin J McAnulty, Shahin Moledina, Adam M Silverstein, Brendan J Whittle, Lucie H Clapp
Prostacyclins are extensively used to treat pulmonary arterial hypertension (PAH), a life-threatening disease involving the progressive thickening of small pulmonary arteries. Although these agents are considered to act therapeutically via the prostanoid IP receptor, treprostinil is the only prostacyclin mimetic that potently binds to the prostanoid EP₂ receptor, the role of which is unknown in PAH. We hypothesised that EP₂ receptors contribute to the anti-proliferative effects of treprostinil in human pulmonary arterial smooth muscle cells (PASMCs), contrasting with selexipag, a non-prostanoid selective IP agonist...
August 12, 2018: International Journal of Molecular Sciences
Ketul R Chaudhary, Yupu Deng, Colin M Suen, Mohamad Taha, Thomas H Petersen, Shirley H J Mei, Duncan J Stewart
BACKGROUND AND PURPOSE: Pulmonary arterial hypertension (PAH) is a life-threatening disease that leads to progressive pulmonary hypertension, right heart failure and death. Parenteral prostaglandins (PGs), including treprostinil, a prostacyclin analogue, represent the most effective medical treatment for severe PAH. We investigated the effect of treprostinil on established severe PAH and underlying mechanisms using the rat SU5416 (SU, a VEGF receptor-2 inhibitor)-chronic hypoxia (Hx) model of PAH...
October 2018: British Journal of Pharmacology
Yangfang Xiang, Jun Li, Yinxiang Sun
RATIONALE: Treprostinil, a potent vasodilator, is the treatment of choice for severe pulmonary arterial hypertension (PAH) during pregnancy. Its inhibition of platelet aggregation increases the risk of hemorrhage. In addition, anticoagulation therapy is widely used in pregnancy with PAH due to the hypercoagulable state. However, very little is known about the complications of anticoagulants' use in pregnancy with PAH. PATIENT CONCERNS: A 27-year-old pregnant woman was admitted to the hospital at 32weeks with progressive dyspnea...
August 2018: Medicine (Baltimore)
Susanna Desole, Manuel Jonas Richter, Alexander Heine, Ralf Ewert
No abstract text is available yet for this article.
January 1, 2018: Pulmonary Circulation
Tobias Gessler
Pulmonary arterial hypertension (PAH) is a rare, but severe and life-threatening disease characterized by vasoconstriction and remodeling of the pulmonary arterioles, leading to progressive increase in pulmonary vascular resistance and ultimately to right-heart failure. In the last two decades, significant progress in treatment of PAH has been made, with currently 12 drugs approved for targeted therapy. Among these, the stable prostacyclin analogues iloprost and treprostinil have been repurposed for inhalation...
June 8, 2018: Advanced Drug Delivery Reviews
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