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PHCentral News is gathered daily to bring you the latest developments in pulmonary hypertension treatments and research as well as stories we think are useful on an array of topics.  Under current news, you will find a dynamic list of stories for the last thirty (30) days.  You will find older news stories in our archives.    Stories about specific treatments can also be found in the medical section.

If you run across a PH news story we've missed or important story with broad appeal, please send us the link and we'll make sure it gets added.   Send news:  contact/info+phcentral+org.



  • January 29, 2015
  • SERAPHIN: Macitentan linked to decreased hospitalization risk
  • (Healio) Treatment with a 10-mg dose of macitentan significantly decreased the risk for and prevalence of all-cause hospitalization among patients with symptomatic pulmonary arterial hypertension, according to findings from the SERAPHIN study.
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  • January 28, 2015
  • Why Sleep Soothes The Flu
  • (Inside Science) A special protein enhances sleep’s healing power and speeds flu recovery. 
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  • Takayasu Arteritis-associated Pulmonary Hypertension
  • (The Journal of Rheumatology) Patients with TA are at increased risk for PH. Early screening of patients with TA with unexplained symptoms related to PH should be applied. PH-specific therapies or revascularization may be effective treatments in the early stages of patients with PA, PH, and severe pulmonary artery stenosis.
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  • January 27, 2015
  • A comparison of the predictive accuracy of three screening models for AH in systemic sclerosis
  • (7th Space) There is evidence that early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcomes. We compared the predictive accuracy of two recently published screening algorithms (DETECT 2013 and Australian Scleroderma Interest Group (ASIG) 2012) for SSc-associated PAH (SSc-PAH) with the commonly used European Society of Cardiology/Respiratory Society (ESC/ERS 2009) guidelines.
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  • January 26, 2015
  • Peripheral muscles weakened in PAH patients
  • (News Medical) Patients with pulmonary arterial hypertension (PAH) have peripheral muscle weakness that is independent of impairments in their respiratory muscles, research shows.
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  • January 25, 2015
  • The Flavonoid Quercetin Reverses Pulmonary Hypertension in Rats
  • (Plos One) Quercetin is a dietary flavonoid which exerts vasodilator, antiplatelet and antiproliferative effects and reduces blood pressure, oxidative status and end-organ damage in humans and animal models of systemic hypertension. We hypothesized that oral quercetin treatment might be protective in a rat model of pulmonary arterial hypertension. Three weeks after injection of monocrotaline, quercetin (10 mg/kg/d per os) or vehicle was administered for 10 days to adult Wistar rats. Quercetin significantly reduced mortality. In surviving animals, quercetin decreased pulmonary arterial pressure, right ventricular hypertrophy and muscularization of small pulmonary arteries. Classic biomarkers of pulmonary arterial hypertension such as the downregulated expression of lung BMPR2, Kv1.5, Kv2.1, upregulated survivin, endothelial dysfunction and hyperresponsiveness to 5-HT were unaffected by quercetin. Quercetin significantly restored the decrease in Kv currents, the upregulation of 5-HT2A receptors and reduced the Akt and S6 phosphorylation. In vitro, quercetin induced pulmonary artery vasodilator effects, inhibited pulmonary artery smooth muscle cell proliferation and induced apoptosis. In conclusion, quercetin is partially protective in this rat model of PAH. It delayed mortality by lowering PAP, RVH and vascular remodeling. Quercetin exerted effective vasodilator effects in isolated PA, inhibited cell proliferation and induced apoptosis in PASMCs. These effects were associated with decreased 5-HT2A receptor expression and Akt and S6 phosphorylation and partially restored Kv currents. Therefore, quercetin could be useful in the treatment of PAH.
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  • Idiopathic PA Hypertension Responds Variably to Vasodilators
  • (Medscape) Patients with idiopathic pulmonary arterial hypertension (IPAH) show different vascular responses to acute vasodilator challenge, according to a small study in Canada.

    "Not all patients with seeming IPAH are alike," Dr. David Langleben, of McGill University, Montreal, Quebec, told Reuters Health by email. "The acute vasodilator responders are a truly distinct group. This group should probably be a separate subcategory in the classification of IPAH."
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  • January 24, 2015
  • CT measures can refine PAH prognosis
  • (News Medical) Structural features identifiable on computed tomography (CT) pulmonary angiography can provide prognostic information for patients with pulmonary arterial hypertension (PAH), say UK researchers.
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  • Early Screening Methods for Pulmonary Arterial Hypertension in Systemic Sclerosis Evaluated in New Study
  • (Pulmonary Hypertension News) A team of researchers from Australia recently published a study, entitled “A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis,” in the journal Arthritis Research & Therapy.
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  • January 23, 2015
  • New Treatments Haven't Lowered Anesthesia Risks for Children with Pulmonary Hypertension
  • (Newswire) Younger Age and More Severe PHT Are Risk Factors for Anesthesia-Related Complications
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  • January 21, 2015
  • Asthma Linked to New-Onset Sleep Apnea
  • (Medscape) Adults with asthma are at increased risk for new-onset obstructive sleep apnea (OSA), according to a new study.
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  • January 20, 2015
  • Seasonal Influenza Vaccine Only 23% Effective This Year
  • (Medscape) An early analysis of the 2014 to 2015 influenza season notes that two thirds of the circulating influenza A (H3N2) viruses differ from the H3N2 component of the 2014 to 2015 influenza vaccine. The 2014 to 2015 seasonal influenza vaccine is thus estimated to be only 23% effective in preventing medically attended, laboratory-confirmed influenza H3N2 infection in the overall population. Vaccine effectiveness against H3N2 viruses is estimated at just 12% for individuals aged 18 to 49 years.
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  • January 19, 2015
  • Ascendis to Start Phase 1 Study of PAH Treatment
  • (Pulmonary Hypertension News) Ascendis Pharma A/S is beginning its phase 1 single ascending dose study on healthy participants to evaluate a potential treatment for pulmonary arterial hypertension (PAH) called TransCon Treprostinil, as announced in a press release. The investigational therapy is one of a few promising drug candidates in the clinical stage biotechnology company’s portfolio, all of which use its novel TransCon technology to respond to the needs of patients who have diseases for which there is no cure, such as PAH.
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  • January 18, 2015
  • Notch Activation of Ca2+ Signaling Mediates Hypoxic Pulmonary Vasoconstriction and Pulmonary Hypertension.
  • (PubMed) Hypoxic pulmonary vasoconstriction (HPV) is an important physiological response that optimizes the ventilation/perfusion ratio. Chronic hypoxia causes vascular remodeling, which is central to the pathogenesis of hypoxia-induced pulmonary hypertension (HPH). We have previously shown that Notch3 is upregulated in HPH, and activation of Notch signaling enhances store-operated Ca2+ entry (SOCE), an important mechanism that contributes to pulmonary arterial smooth muscle cell (PASMC) proliferation and contraction.
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  • Pacific Therapeutics CEO Provides Updates About Therapy For Idiopathic Pulmonary Fibrosis
  • (Pulmonary Hypertension News) Thanks to a partnership with IntelGenx Corp (IGXT), Pacific Therapeutics Ltd. is combining two approved drugs to create a once per day tablet to treat pulmonary fibrosis and other diseases that result from excess scarring.
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  • Bellerophon Therapeutics (BLPH) Files $69M IPO
  • (StreetInsider) Bellerophon Therapeutics is a clinical-stage therapeutics company focused on developing innovative products at the intersection of drugs and devices that address significant unmet medical needs in the treatment of cardiopulmonary and cardiac diseases. The company has two programs in advanced clinical development. The first program, INOpulse, is based on their proprietary pulsatile nitric oxide delivery device. They are currently developing two product candidates under the INOpulse program: one for the treatment of pulmonary arterial hypertension, or PAH, for which they intend to commence Phase 3 clinical trials in the second half of 2015, and the other for the treatment of pulmonary hypertension associated with chronic obstructive pulmonary disease, or PH-COPD, which is in Phase 2 development. The second program is bioabsorbable cardiac matrix, or BCM, which is currently in a placebo-controlled clinical trial designed to support CE mark registration in the European Union. The company completed enrollment of this trial in December 2014, with 303 patients having completed the treatment procedure, and expect to report top line results in mid-2015. Assuming positive results, they intend to conduct a pivotal pre-market approval trial of BCM beginning in the first half of 2016, which will be designed to support registration in the United States. They are developing BCM for the prevention of cardiac remodeling, which often leads to congestive heart failure following an ST-segment elevated myocardial infarction, or STEMI.
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  • January 17, 2015
  • Effects of Epoprostenol on Right Ventricular Hypertrophy and Dilatation in Pulmonary Hypertension
  • (Life Without Diseases) Primary pulmonary hypertension (PPH) is a rare, progressive, and fatal disease. Continuous administration of epoprostenol improves survival and exercise tolerance in patients with PPH. Clinical experience shows that most of the improvement takes place in the first few months of treatment. The mechanism underlying this improvement is not yet fully understood.
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  • Exercise Pathophysiology in Patients With Primary Pulmonary Hypertension
  • (Circulation) Patients with primary pulmonary hypertension (PPH) have a pulmonary vasculopathy that leads to exercise intolerance due to dyspnea and fatigue. To better understand the basis of the exercise limitation in patients with PPH, cardiopulmonary exercise testing (CPET) with gas exchange measurements, New York Heart Association (NYHA) symptom class, and resting pulmonary hemodynamics were studied.
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  • January 16, 2015
  • Pulmonary Arterial Hypertension Medications Studied for Drug Interactions
  • (Pulmonary Hypertension News) According to a study published by researchers at Novartis Pharma, common medications for severe pulmonary arterial hypertension interact, increasing one another’s levels when taken at the same time. However, co-administration of the medications does not appear to impact their effectiveness or increase the risk of liver problems. The study, entitled, “Pharmacokinetic interactions among imatinib, bosentan and sildenafil, and their clinical implications in severe pulmonary arterial hypertension,” appeared in the January 7th issue of the British Journal of Clinical Pharmacology.
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  • Pulmonary hypertension in idiopathic pulmonary fibrosis does not influence six-minute walk distance
  • (Pubmed) The characteristics of the six-minute walk test (6MWT) in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary hypertension (PH) have not yet been described; nevertheless, this test has already been used as a "surrogate end point" in some clinical trials.Objective: Goal of this retrospective study was to assess whether the presence of PH in patients with IPF might influence 6MWT performances.
    [..]
    Conclusions: Our data suggested that 6MWD does not differ between IPF patients with or without PH. Thus, 6MWD should not be used as a surrogate endpoint in clinical study in patients affected by IPF and PH.
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  • January 15, 2015
  • Reducing TRPC1 Expression through Liposome-Mediated siRNA Delivery Markedly Attenuates Hypoxia-Induced Pulmonary Arterial Hypertension in a Murine Model.
  • (Pubmed) Experiment showing that delivery of siRNA that controls gene expression of TPRC1, a transient receptor channel, involved in hypoxic PAH is possible. At least in mice.
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  • January 14, 2015
  • Ascendis Pharma A/S Announces Initiation of a Phase 1 Single Ascending Dose Study of TransCon Treprostinil
  • (Prnewswire.com) COPENHAGEN, Denmark, Jan. 14, 2015 /PRNewswire/ -- Ascendis Pharma A/S, a clinical stage biotechnology company that applies its innovative TransCon technology to address significant unmet medical needs, today announced that it has initiated a Phase 1 single ascending dose study of TransCon Treprostinil in healthy volunteers. TransCon Treprostinil is being developed to treat pulmonary arterial hypertension, a life-threatening orphan disease characterized by elevated blood pressure in the pulmonary arteries.

    TransCon Treprostinil has been designed as an inert prodrug that enables a sustained release of unmodified treprostinil in circulation following a once-daily self-administered subcutaneous injection. TransCon Treprostinil is intended to produce a pharmacokinetic profile comparable to continuously infused prostacyclins, with the benefit of a more convenient route of administration, while avoiding the infusion site pain and risk of bloodstream infections associated with currently marketed infused prostacyclin therapies...
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  • Survival of adults with systemic sclerosis following lung transplantation: A nationwide cohort study.
  • (Arthritis Rheumatology) A diagnosis of SSc may confer an increased risk of death 1 year following lung transplantation compared to a diagnosis of ILD, but this risk is similar to that of PAH, a widely accepted indication for lung transplantation.
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  • January 13, 2015
  • Pharmacokinetic interactions among imatinib, bosentan and sildenafil, and their clinical implications in severe pulmonary arterial hypertension.
  • (PubMed) Population plasma concentrations of imatinib in patients with severe PAH were found comparable with those of patients with chronic myeloid leukaemia. Imatinib was found effective regardless of the co-medications and showed intrinsic efficacy beyond merely elevating the concentrations of the co-medications due to DDIs. There was no evidence of increased risk of liver toxicity upon co-administration with bosentan, despite an incrase of bosentan's plasma concentration and 64% increase of sildenafil PK.

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  • January 12, 2015
  • Arena Pharmaceuticals Initiates Phase 2 Clinical Trial Evaluating Ralinepag for Pulmonary Arterial Hypertension
  • (Pipelinereview.com) SAN DIEGO, CA, USA I January 12, 2015 I Arena Pharmaceuticals, Inc. (NASDAQ: ARNA) announced today the initiation of patient dosing in a Phase 2 clinical trial of ralinepag, an oral, non-prostanoid prostacyclin (IP) receptor agonist intended for the treatment of pulmonary arterial hypertension (PAH).

    "An intriguing component of this compound is the smooth peak-to-trough ratio, which may potentiate efficacy and minimize toxicity relative to currently available therapies," said Lewis J. Rubin, M.D., Emeritus Professor of Medicine at University of California, San Diego, School of Medicine. "This clinical trial should help to further elucidate how ralinepag's intrinsic properties translate to its efficacy and safety profile."

    This 22-week, randomized, double-blind and placebo-controlled Phase 2 trial will..
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  • January 11, 2015
  • Pulmonary Hypertension in Chronic Lung Diseases
  • (Journal of the American College of Cardiology) Chronic obstructive lung disease (COPD) and diffuse parenchymal lung diseases (DPLD), including idiopathic pulmonary fibrosis (IPF) and sarcoidosis, are associated with a high incidence of pulmonary hypertension (PH), which is linked with exercise limitation and a worse prognosis. Patients with combined pulmonary fibrosis and emphysema (CPFE) are particularly prone to the development of PH. Echocardiography and right heart catheterization are the principal modalities for the diagnosis of COPD and DPLD. For discrimination between group 1 PH patients with concomitant respiratory abnormalities and group 3 PH patients (PH caused by lung disease), patients should be transferred to a center with expertise in both PH and lung diseases for comprehensive evaluation.
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  • Role of BNP and Echo measurement for pulmonary hypertension recognition in patients with interstitial lung disease: an algorithm application model
  • (Respiratory Medicine Journal) This study evaluated the role of echocardiography and BNP in patients with interstitial lung disease (ILD), to identify those with PH and RV dysfunction. The aims of this study were: 1-to evaluate the accuracy of an algorithm including BNP, DLCO and echocardiographic measurements to identify PH and RV dysfunction; 2- to evaluate BNP and Echo values concordance in relation to right catheterization measurement.
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  • January 10, 2015
  • United Therapeutics and DEKA Sign Agreement for New PAH Remodulin Delivery System
  • (Pulmonary Hypertension News) United Therapeutics Corporation has signed an agreement with DEKA Research & Development Corp., to continue the development of a breakthrough technology for the subcutaneous delivery of the Remodulin (treprostinil) Injection. The device is expected to offer a potential treatment option for patients who suffer from pulmonary arterial hypertension (PAH) and improve administration of Remodulin.
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  • Modified Potts Shunt in an adult with pulmonary arterial hypertension and recurrent syncope – three-year follow-up
  • (International Journal of Cardiology) Creation of a Potts anastomosis (left pulmonary artery to descending aorta) has sometimes been used in children to manage severe pulmonary hypertension (PH), refractory to maximal medical treatment, in patients with severe symptoms including syncope. The attraction of this approach is that in natural history studies of patients with PH, those with equal ventricular pressures (e.g. Eisenmenger circulation in the context of a ventricular septal defect) may have a good quality of life and prolonged survival compared to those with end-stage PH and episodic supra-systemic right ventricular hypertension.
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  • January 8, 2015
  • Flu Season 2015: Influenza In US ‘Widespread,’ But Fear May Outpace Threat
  • (International Business Times) With an unforeseen mutation in this year’s dominant influenza virus and a faulty vaccine, 43 states have seen “widespread” transmission of the flu this season, according to the latest report from the U.S. Centers for Disease Control and Prevention. Flu activity was rampant in 29 states during the final week of December, up from 22 states a week earlier. Twenty-one pediatric deaths have been reported.
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  • January 7, 2015
  • Could a National PH Registry Improve Research and Treatment in America?
  • (Pulmonary Hypertension News) Initial results are now available from a national registry that has been established in Russia to track individuals with pulmonary hypertension (PH), including chronic thromboembolic pulmonary hypertension (CTEPH). The research was published in Russian in the journal Terapevticheskii Arkhiv (translated to Therapeutic Archive).
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  • January 6, 2015
  • Health Talk: Know the signs and symptoms of COPD
  • (Capital Gazette) Chronic obstructive pulmonary disease refers to a group of lung diseases that block airflow and make breathing difficult. Emphysema and chronic bronchitis are the two most common conditions that make up COPD.
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  • January 5, 2015
  • Pulmonary Arterial Hypertension in the US: How Much Does it Cost?
  • (Lung Disease News) A recent study published in BMC Health Services Research attempted to answer this question, by estimating resource utilization and health care costs caused by PAH in a large US managed care health plan.
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  • January 4, 2015
  • Diagnosis, Assessment, and Treatment of Non-Pulmonary Arterial Hypertension Pulmonary Hypertension
  • (Research Gate) Previous international meetings on pulmonary hypertension (PH) have focused predominantly on pulmonary arterial hypertension (PAH), a form of PH that is usually severe but overall quite rare. The 4th World Symposium was the first to assign a working group to address in detail the so-called non-PAH forms of PH, that is, those forms of PH that are encountered in patients with chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), left heart disease (LHD), venous thromboembolism, and other conditions. It is a paradox in the field of PH that one of the less common forms, that is, PAH, has been extensively studied, whereas fewer data are available on other types of PH, many of which are far more common. At the same time, drugs with proven efficacy in PAH (1–3) are being increasingly used in other forms of PH, despite the virtual absence of clinical trials supporting this approach.
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  • Medtronic Submits Drug Delivery System to FDA to Complement United Therapeutics’ PAH Treatment
  • (Lung Disease News) Medical technology developer Medtronic, Inc. recently completed an application submission to the U.S. Food and Drug Administration (FDA) regarding the pre-market approval of their SynchroMed II implantable drug infusion system, which includes a new catheter design. The system is intended to be used by pulmonary arterial hypertension (PAH) patients for the intravenous delivery of the drug Remodulin (treprostinil), which is being developed by the United Therapeutics Corporation.
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  • January 3, 2015
  • Actelion Submits New Drug for Pulmonary Arterial Hypertension to FDA
  • (Lung Disease News) Actelion has submitted its new treatment for pulmonary arterial hypertension (PAH) to the U.S. Food and Drug Administration (FDA). The company is applying for a New Drug Application (NDA) for selexipag (Uptravi), after having also submitted the drug to the European Medicines Agency (EMA).
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  • Absence of the calcium-binding protein, S100A1, confers pulmonary hypertension in mice associated with endothelial dysfunction and apoptosis
  • (Oxford Journals) S100A1, a 10-kDa, Ca2+-binding protein, is expressed in endothelial cells (ECs) and binds eNOS. Its absence is associated with impaired production of nitric oxide (NO) and mild systemic hypertension. As endothelial dysfunction contributes to clinical and experimental pulmonary hypertension (PH), we investigated the impact of deleting S100A1 in mice, on pulmonary haemodynamics, endothelial function, NO production, associated signalling pathways, and apoptosis.
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