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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.



  • May 27, 2016
  • Breakthrough could help sufferers of fatal lung disease
  • (Medical Xpress) For the first time scientists have identified a molecule that promotes the progression of the disease which affects 6,500 people in the UK.
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  • May 26, 2016
  • The Reviva Pharmaceuticals results were presented at the American Thoracic Society International Conference 2016
  • (Lung Disease News) RP5063 is a potent inhibitor of serotonin 5-HT receptor, a novel target for the treatment of PAH. The orally active new chemical showed positive efficacy results in the treatment of PAH because it was found to reduce the mean pulmonary arterial pressure, decrease respiratory resistance, and bring blood oxygen levels back to normal.
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  • May 25, 2016
  • PAH Drug, Riociguat, Found Not to Alter the Effectiveness of Oral Contraceptives
  • (Pulmonary Hypertension News) Women with PAH are advised to avoid having children due to the high mortality rate associated with pregnancy in this condition. Since oral contraceptives are the most common drugs used to prevent pregnancy, it is important to understand how they interact with a drug used to treat PAH.
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  • Speaker: Treatment of systemic lupus erythematosus should be flexible
  • (Healio) Treatment of systemic lupus erythematosus should fluctuate based on the affected organs and severity of the condition, Janet Pope, MD, MPH, professor of medicine, epidemiology and biostatistics at the University of Western Ontario Schulich School of Medicine in London, Ontario, said at the Congress of Clinical Rheumatology Annual Meeting.
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  • May 24, 2016
  • Mast Therapeutics (MSTX) Reports Positive Interim Results From Second Phase 2a Study of AIR001 in Patients With HFpEF
  • (Street Insider) In the 10 patients who had been studied to date, nebulized inhaled nitrite (AIR001) administration significantly lowered central pressures, specifically, right atrial, right ventricular systolic and diastolic, pulmonary artery (PA) systolic/diastolic/mean, and pulmonary artery occlusion (PAOP) pressures. Of note, pulmonary artery occlusion and mean pulmonary artery pressures were markedly decreased from baseline median values. In addition, there was an observed increase in pulmonary artery compliance. There was no significant decrease is systemic blood pressures or change in heart rate. Methemoglobin levels increased modestly, but remained less than 1.9% and did not meet stopping criteria of the study, which was 5%. AIR001 was generally well-tolerated.
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  • May 23, 2016
  • MEF2C-MYOCD and Leiomodin1 Suppression by miRNA-214 Promotes Smooth Muscle Cell Phenotype Switching in PAH
  • (PLOS One) Vascular hyperproliferative disorders are characterized by excessive smooth muscle cell (SMC) proliferation leading to vessel remodeling and occlusion. In pulmonary arterial hypertension (PAH), SMC phenotype switching from a terminally differentiated contractile to synthetic state is gaining traction as our understanding of the disease progression improves. While maintenance of SMC contractile phenotype is reportedly orchestrated by a MEF2C-myocardin (MYOCD) interplay, little is known regarding molecular control at this nexus. Moreover, the burgeoning interest in microRNAs (miRs) provides the basis for exploring their modulation of MEF2C-MYOCD signaling, and in turn, a pro-proliferative, synthetic SMC phenotype. We hypothesized that suppression of SMC contractile phenotype in pulmonary hypertension is mediated by miR-214 via repression of the MEF2C-MYOCD-leiomodin1 (LMOD1) signaling axis.
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  • May 21, 2016
  • Vascular BioSciences Presents New Data from Pulmonary Hypertension Studies at ATS 2016
  • (Pulmonary Hypertension News) The first abstracts presented, “Oral CAR Peptide Adjuvant Enhances Sildenafil Therapy for Pulmonary Arterial Hypertension” and “CAR Peptide Adjuvant Enables Effective Pulmonary Hypertension Treatment with Imatinib at Reduced Doses,” were part of a collaborative effort between Vascular and Dr. Masanobu Komatsu and colleagues at the Sanford Burnham Prebys Medical Discovery Institute  in Lake Nona, Florida.
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  • May 20, 2016
  • Safety, efficacy, and clinical utility of macitentan in the treatment of pulmonary arterial hypertension
  • (Dove Press) Until relatively recently, the treatment options for those afflicted by pulmonary arterial hypertension were limited; today, a greater understanding of the pathophysiology behind this disease has led to several evidence-based therapies that can improve pulmonary function and quality of life for these patients.
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  • May 19, 2016
  • Actelion gets EU go-ahead for Uptravi in PAH
  • (PM Live) The Swiss biopharma company rates Uptravi (selexipag) as one of the most important drugs in its pipeline as it deals with competition to older products in its PAH franchise such as Tracleer (bosentan).
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  • May 18, 2016
  • US trial tests machine that 'reconditions' lungs outside body
  • (Fox Health) The XPS, manufactured by Swedish company XVIVO Perfusion, is in clinical trials at 16 U.S. medical centers. Known as "the box," it ventilates the lungs after removal from the donor and infuses them with a fluid mix of drugs and steroids, effectively drying them out and getting them in better shape for use in a transplant operation.
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  • May 17, 2016
  • Bayer Stops Phase II Adempas Study Due to Safety Issues
  • (Zacks) The decision followed the recommendation of an independent Data Monitoring Committee (DMC), which observed that patients dosed with Adempas were at a greater risk of death and other serious adverse events than those on the placebo arm.
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  • May 16, 2016
  • PDE4 Inhibitor Identified in Study as New and Promising Treatment for Pulmonary Hypertension
  • (Pulmonary Hypertension News) The phosphodiesterase (PDE) 4 inhibitor, GPD1116, effectively treats pulmonary hypertension and several other diseases in animal models, and holds promise as a new therapeutic drug, researchers reported in the study, “Pharmacological profile of GPD-1116, an inhibitor of phosphodiesterase 4,” published in the Biological and Pharmaceutical Bulletin.
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  • May 15, 2016
  • More evidence for combination therapy benefits in PAH
  • (News Medical) A meta-analysis published last month found a reduced risk of clinical worsening among patients given combination therapy. And the latest meta-analysis, published in CHEST, shows benefits for a range of individual clinical indicators, including 6-minute walk distance, functional class and haemodynamics.
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  • May 14, 2016
  • Detailed digital human models could hold key to future clinical research
  • (Medical Xpress) Dr Swift and his team have developed a non-invasive test that uses MRI scan data to quantify changes that occur in the heart and provide an equivalent pressure reading. His model combines data on hypertrophy (the thickening of the heart muscle of the right ventricle), distortion of the septum (the wall which separates the right and left ventricles), distortion of the artery and the forward and backward flow waves in the pulmonary artery.
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  • May 13, 2016
  • Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis
  • (Respiratory Medicine Case Reports) Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by the infiltration of large numbers of Langerhans cells to the involved tissues and is a rare lung disease that occurs predominantly in young smokers. The clinical course and prognosis of PLCH are unpredictable. Although most patients with PLCH recover spontaneously or remain in a stable condition without treatment after smoking cessation, some patients experience progressive respiratory impairment related to a deleterious change in lung function or to the development of pulmonary arterial hypertension (PAH). Precapillary PAH is an important complication in patients with PLCH, since PAH is associated with a poor prognosis. However, little information on the optimal treatment of PAH caused by PLCH (PAH-PLCH) is available. Indeed, the limited data available for PAH-PLCH suggests that PAH-specific therapies are associated with a long term improvement in hemodynamics.
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  • May 12, 2016
  • HIPPO-integrin Linked Kinase Crosstalk Controls Self-sustaining Proliferation and Survival in Pulmonary Hypertension
  • (ATS Journals) These data report inactivation of HIPPO/LATS1, self-supported via Yap-fibronectin-ILK1 signaling loop, as a novel mechanism of self-sustaining proliferation and apoptosis resistance of PAVSMC in PAH and suggest a new potential target for therapeutic intervention.
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  • May 11, 2016
  • Chronic thromboembolic pulmonary hypertension - a diagnostic and therapeutic update.
  • (PubMed) Chronic thromboembolic pulmonary hypertension (CTEPH), an important cause of severe pulmonary hypertension, is still underdiagnosed, mainly due to the insufficient use of V/Q scannning in patients with pulmonary hypertension. This article reviews the current diagnostic approach and discusses the therapeutic options in this particular form of pulmonary hypertension. Every patient with CTEPH should undergo an evaluation in a specialised centre with experience in pulmonary arterial endarteriectomy (PEA) as the potentially curative surgical technique. Partly unresolved questions regard the status of the recently described percutaneous transluminal pulmonary angioplasty and the best medical treatment in patients with inoperable or recurrent/persistent pulmonary hypertension after PEA.
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  • May 10, 2016
  • AVEO Announces Filing of Provisional Patent Applications for AV-353, a Notch 3-Specific Inhibitor Antibody for PAH
  • (Business Wire) Current treatments in PAH focus only on controlling symptoms by avoiding vasoconstriction and increasing vasodilation of vessels and do not reverse the underlying cause of the disease. In contrast, with the results of a recently concluded research study supported by AVEO, AV-353 has generated a growing body of preclinical data that supports AV-353’s ability to potentially reverse the disease phenotype, which would represent a potential disease-modifying approach to treatment. A manuscript of the results is being prepared for submission to a peer-reviewed journal.
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  • May 9, 2016
  • Older Lung Donors Raise Mortality Risk Only Slightly
  • (Medscape) For people who undergo lung transplantation, mortality risk is only slightly higher when donors are 55 to 64 years of age than when they are younger than 55 years, the current age recommendation, according to an analysis of the world's largest lung transplant registry.
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  • May 8, 2016
  • Cumberland Pharmaceuticals Announces New Program To Develop Vasculan™ For Systemic Sclerosis
  • (Street Insider) Cumberland Pharmaceuticals Inc. (NASDAQ: CPIX), a specialty pharmaceutical company focused on the development of innovative treatments for underserved patient populations, announced today the addition of VASCULAN TM to its pipeline. Cumberland has initiated the clinical development of VasculanTM (ifetroban) oral capsule for the treatment of systemic sclerosis. The U.S. Food and Drug Administration (FDA) has cleared Cumberland's investigational new drug application (IND) for Phase II clinical program for Vasculan in patients with systemic sclerosis.
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  • May 7, 2016
  • Effect of dual pulmonary vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease
  • (PubMed) For the majority of patients, monotherapy with a PAH-specific medication provided improved and sustained exercise benefits. For the small percentage of patients who required it, add-on therapy appeared to prevent further deterioration in exercise capacity but did not improve 6MWD.
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  • May 6, 2016
  • Aerosol-Delivered Gene Therapy Seen to Stop Vascular Remodeling in PH Study in Pigs
  • (Pulmonary Hypertension News) Researchers from Mount Sinai, and their collaborators in Boston and Spain, first aimed to understand whether it was feasible to deliver the SERCA2a gene to the narrowed blood vessels of the lung by aerosol administration of an engineered adeno-associated virus that carried the gene.
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  • May 5, 2016
  • Right Ventricle Enlargement Successfully Treated in PAH Animal Model Using Ranolazine, a Heart Drug
  • (Pulmonary Hypertension News) Researchers from Chungbuk National University, Korea, found that ranolazine (Ranexa), a drug used to treat coronary heart disease, effectively reduced the structural remodeling of the heart’s right ventricle in an animal model of pulmonary arterial hypertension (PAH). The results suggested that targeting right ventricle disease could delay PAH progression in people.
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  • May 4, 2016
  • Right Ventricle Dysfunction in PAH May Be Treated by Drug Targeting a Signaling Pathway Independent of Arterial Pressure
  • (Pulmonary Hypertension News) Scientists have long believed that right ventricle dysfunction in PAH patients is a compensatory mechanism caused by increasing pressure in lung arteries. Enlargement of the heart’s right ventricle eventually leads to right ventricle failure, the most common cause of death for PAH patients. While the mechanisms behind the enlargement are not clear, the death of heart muscle cells is suggested to be involved.
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  • Tissue-engineered artificial lung that mimics the response of the human lung to drugs, toxins and other agents
  • (Medical Xpress) Scientists at Los Alamos National Laboratory are developing a miniature, tissue-engineered artificial lung that mimics the response of the human lung to drugs, toxins and other agents.
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  • May 3, 2016
  • Pediatric research: Researchers now think fibrosis can be reversed
  • (The Columbus Dispatch) Fibrosis, one of the most devastating consequences of hundreds of human diseases, has long been thought by biomedical science to be fixed and irreversible.
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  • PAH Is Rising in Elderly, and So Are Challenges in Correctly Diagnosing and Treating Their Disease
  • (Pulmonary Hypertension News) Elderly people at increased risk of pulmonary hypertension (PH) are increasingly also being diagnosed with pulmonary arterial hypertension (PAH). Diagnosis and management in this group can be quite challenging, and a report from Geneva University Hospitals suggested that all elderly patients with suspected PAH should be referred to an expert center for proper diagnosis and treatment of a disease that can have devastating consequences.
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  • May 2, 2016
  • Scientists Discover Shared Genes in COPD and IPF, Pointing to Potential Drug Development
  • (Lung Disease News) Despite sharing some risk factors, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are usually studied in isolation, and the presence of possible shared molecular mechanisms has been unknown.
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  • Special training strengthens heart of patients with pulmonary hypertension: study
  • (Shanghai Daily) A customized exercise program for patients with pulmonary hypertension could improve their oxygen uptake and heart strength, said a study of University Hospital Heidelberg on Wednesday. 
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  • May 1, 2016
  • Yoga Shows Promise as Way to Improve Quality of Life in Pulmonary Arterial Hypertension (PAH) Patients
  • (Globe Newswire) WASHINGTON, April 28, 2016 (GLOBE NEWSWIRE) -- Today at the 36th Annual International Society for Heart and Lung Transplantation (ISHLT) Meeting & Scientific Sessions, researchers from the Universities of Calgary and Alberta in Canada presented findings from a study testing the effects of Iyengar Yoga (IY) on patients suffering from varying stages of Pulmonary Arterial Hypertension (PAH). The study was aimed at determining if two-hour IY sessions over 12 weeks would improve health-related quality of life, particularly in patients suffering anxiety and depression.
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  • Drugs for Pulmonary Arterial Hypertension: Comparative Efficacy, Safety, and Cost-Effectiveness
  • (PubMed) The objective of this therapeutic review is to conduct a systematic review to assess the comparative efficacy and safety and to determine the cost-effectiveness of drug therapies for the treatment of PAH in adults.
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  • April 30, 2016
  • Gene therapy halts pulmonary hypertension progression in large animal pre-clinical study
  • (Medical Xpress) Scientists have used a novel gene therapy to halt the progression of pulmonary hypertension, a form of high blood pressure in the lung blood vessels that is linked to heart failure, according to a study led by Roger J. Hajjar, MD, Professor of Medicine and Director of the Cardiovascular Research Center at the Icahn School of Medicine at Mount Sinai. The study was published online this week, and will appear in the May 3 issue of the Journal of the American College of Cardiology (JACC). 
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  • TGA approves combination treatment for PAH treatment
  • (The Pharma Letter) Australian regulator the Therapeutic Goods Administration (TGA) has approved a variation to expand the current therapeutic indication for Volibris (ambrisentan) to include its use in combination treatment with tadalafil for patients with pulmonary arterial hypertension (PAH) in Australia.
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  • April 29, 2016
  • Memorial Hermann Heart & Vascular Institute-Texas Medical Center Launches Lung Transplant Program
  • (PR Newswire) Memorial Hermann-Texas Medical Center (TMC) is now offering lung transplants, establishing its reputation as an elite health center with a comprehensive transplant program covering all solid organs including kidney, liver and heart transplants.
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  • April 28, 2016
  • Minimally important dyspnoea, fatigue changes in PAH patients defined
  • (News Medical) Researchers say that a change of around 1 unit in Borg dyspnoea or fatigue scores signifies an important change in patients with pulmonary arterial hypertension (PAH)
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