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.

  • August 20, 2014
  • Endothelin@25 - new agonists, antagonists, inhibitors and emerging research frontiers: IUPHAR Review 12.
  • (British Journal of Pharmacology) 25 years of Endothelin research in review and outlook
  • Novel Mechanisms of Sildenafil in Pulmonary Hypertension Involving Cytokines/Chemokines, MAP Kinases and Akt.
  • ( New data suggest a beneficial effect of sildenafil on inflammatory and kinase signaling mechanisms that substantially contribute to its protective effects, and may have potential implications in designing future therapeutic strategies in the treatment of pulmonary hypertension.
  • August 19, 2014
  • Obituary: Dame Julia Polak DBE
  • ( Julia Polak a professor at London's King's College got IPAH diagnosis in the 90ies and got a heart and lung transplant in 1995. After her PH diagnosis she changed her scientific work towars tissue engineering, to build or grow lung tissue.
    Really worth reading.  
  • New Obstructive Sleep Apnea (OSA) Diagnosis Guidelines
  • (Netscape) Patients with unexplained daytime sleepiness should undergo a sleep study, preferably polysomnography (PSG), according to new recommendations developed by the American College of Physicians.

    However, these recommendations for diagnosing obstructive sleep apnea (OSA), based in part on a literature search, are weak, and the quality of the evidence was graded as low or moderate, according to the authors...
  • Boehringer Ingelheim Announces U.S. Filing for the Fixed-Dose Combination Tiotropium Plus Olodaterol for Patients with COPD
  • (Market Watch) RIDGEFIELD, Conn., Aug. 19, 2014 /PRNewswire/ -- Boehringer Ingelheim today announced that the U.S. Food and Drug Administration (FDA) accepted for review the New Drug Application (NDA) for the fixed-dose combination (FDC) of tiotropium and olodaterol delivered via the Respimat® inhaler for the proposed indication of long-term, once-daily maintenance treatment of airflow obstruction in patients with chronic obstructive pulmonary disease (COPD), including chronic bronchitis and/or emphysema. Tiotropium + olodaterol FDC will not be indicated to treat acute deteriorations of COPD or to treat asthma.

    Tiotropium + olodaterol FDC is an investigational treatment consisting of the long-acting muscarinic antagonist (LAMA) tiotropium and the long-acting beta agonist (LABA) olodaterol, and is being evaluated for once-daily use via the Respimat® inhaler. The Respimat® inhaler is a propellant-free inhaler that generates a slow-moving mist.
  • August 18, 2014
  • Phosphodiesterase 5 inhibitors augment UT-15C-stimulated ATP release from erythrocytes of humans with PAH
  • (PubMed) UT-15C-induced ATP release was greater in PAH erythrocytes than in healthy human erythrocytes in both the presence and the absence of PDE5 inhibitors. The finding that prostacyclin analogs and PDE5 inhibitors work synergistically to enhance release of the potent vasodilator ATP from PAH erythrocytes provides a new rationale for the co-administration of these drugs in this disease. Moreover, these results suggest that the erythrocyte is a novel target for future drug development for the treatment of PAH.
  • New Clues From Heart Imaging Give Researchers Insight Into PH
  • (Pulmonary Hypertension News) A team of researchers from the Imperial College London, UK, has preformed a three-dimensional speckle tracking (3D-ST) study and found that patients who have pulmonary hypertension (PH) are found to suffer from reduced right ventricular (RV) strain and notably more dyssynchronous ventricles compared to healthy individuals.
  • August 17, 2014
  • Playing the harmonica for health
  • (STL Today) As people age, the diaphragm muscle weakens and makes it harder to breathe deeply. The rib cage is not as flexible to allow for lung expansion. Alveoli, tiny air sacs in the lungs, lose their elasticity. All of these changes can cause oxygen levels to drop and breathing to become more difficult.
  • August 16, 2014
  • Paediatric PAH treatment goals identified
  • (MedWire News) “Although predictors of survival may be important when starting treatment for PAH, this does not mean that they can be used as treatment goals”, remark Mark-Jan Ploegstra and colleagues from University Medical Center Groningen.
  • Left Ventricle Function Before and After CTEPH-Associated Surgery Assessed In New Study
  • (Pulmonary Hypertension News) A team of researchers has published their results assessing left atrial volume in chronic thromboembolic pulmonary hypertension (CTEPH) patients before and after pulmonary thromboendarterectomy (PTE), a surgical procedure that removes organized, clotted blood from the pulmonary arteries.
  • August 15, 2014
  • Oldies Can Be Goodies: ERA Macitentan Effective in Treating PAH During Unique Trial
  • (Pulmonary Hypertension News) Macitentan was under evaluation in the Phase 3 clinical trial SERAPHIN. The study was unique in that it tested not only 6MWD but also patient-relevant outcomes. Cardiopulmonary haemodynamics, morbidity, and mortality were evaluated during SERAPHIN, and the two doses of macitentan delivered (3 and 10 mg) demonstrated significant improvements in all reported parameters. According to Dr. John Granton, Director of the Pulmonary Hypertension Program at Toronto General Hospital, “Both doses produced a reduction in peripheral vascular resistance and increase in cardiac output compared to placebo.” Dr. Granton spoke in a news article on MedNet, entitled, “Pulmonary Hypertension: New Therapies in Chronic Thromboembolic Pulmonary Hypertension and Pulmonary Arterial Hypertension,” produced after the Annual Congress of the European Respiratory Society.
  • August 14, 2014
  • Pulmonary Hypertension & Pulmonary Arterial Hypertension: Categories, Risk Factors And Treatments Profiled
  • (Pulmonary Hypertension News) Pulmonary Hypertension (PH) and Pulmonary Arterial Hypertension (PAH) are not popular topics of media coverage and popular conversation in the way that Cancer and Heart Disease are. Despite increasing numbers of people diagnosed with PH/PAH worldwide, pulmonary hypertension has been a historically neglected disease. Which is a bit puzzling, since PH/PAH symptoms can be debilitating, initially including shortness of breath and fatigue, but if left untreated, they can lead to right ventricular failure, volume overload, and death. PAH has a high mortality rate, with 60 to 80 percent of patients dying within five years of diagnosis. However, because the molecular processes associated with the disease are difficult to define simply, understanding PH therefore remains enigmatic to most laypeople.
  • August 13, 2014
  • CHEST Posts Free to View Version of PAH Guidelines for Clinicians
  • (Pulmonary Hypertension News) The new guidelines for the management of pulmonary arterial hypertension (PAH) in adults published by The American College of Chest Physicians (CHEST) is free to view in this month’s online issue of the journal CHEST for patients and clinicians alike.
  • Study Shows Patients With Pulmonary Arterial Hypertension Benefit From Specialized Care Centers
  • (Pulmonary Hypertension News) Expert medical specialty care for rare diseases like pulmonary arterial hypertension was recently the subject of review in an article by Sean M. Studer, MD, MS, Chief of Medicine, Woodhull Hospital Medical Center, NY. The article, entitled “Do Patients With Pulmonary Arterial Hypertension Benefit From Referral to a Specialized Center?” and published in the journal Advances in Pulmonary Hypertension, explored the possibility of improved care for patients suffering from PAH in specialized centers, since direct care for patients outside these centers is not common practice. According to the author, “to best support the argument for referral, this anticipated benefit should be evidence-based, include independent accreditation of specialty centers, and offer multiple opportunities to improve outcome and quality of life in PAH.”
  • FDA approves donor lung preservation device that may result in more lung transplants
  • ( Today, the U.S. Food and Drug Administration approved the XVIVO Perfusion System (XPS) with STEEN Solution, a device for preserving donated lungs that do not initially meet the standard criteria for lung transplantation but may be transplantable if there is more time to observe and evaluate the organ’s function to determine whether the lung is viable for transplantation.
  • August 12, 2014
  • How Obesity and Diet Impact COPD
  • (MPR) Abstract: The global increase in the prevalence and incidence of obesity has called serious attention to this issue as a major public health concern. Obesity is associated with many chronic diseases, including cardiovascular disease and diabetes, and recently the role of overweight and obesity in lung disease has received new interest. 
  • Does the Sea Air Have Curative Powers
  • (Wall Street Journal) In the 18th and 19th centuries, ocean air was considered a cure-all for nearly any illness, from depression to tuberculosis. But is there real science to the supposed healing powers of a visit to the sea? 
  • August 11, 2014
  • Pulmonary Hypertension & Pulmonary Arterial Hypertension: Categories, Risk Factors And Treatments Profiled
  • (Pulmonary Hypertension News) Pulmonary Hypertension (PH) and Pulmonary Arterial Hypertension (PAH) are not popular topics of media coverage and popular conversation in the way that Cancer and Heart Disease are. Despite increasing numbers of people diagnosed with PH/PAH worldwide, pulmonary hypertension has been a historically neglected disease. Which is a bit puzzling, since PH/PAH symptoms can be debilitating, initially including shortness of breath and fatigue, but if left untreated, they can lead to right ventricular failure, volume overload, and death. PAH has a high mortality rate, with 60 to 80 percent of patients dying within five years of diagnosis. However, because the molecular processes associated with the disease are difficult to define simply, understanding PH therefore remains enigmatic to most laypeople.
  • August 10, 2014
  • TRAIL Deficient Mice Are Protected from Sugen/Hypoxia Induced Pulmonary Arterial Hypertension
  • (Diseases) Pulmonary arterial hypertension (PAH) is a progressive lung disease diagnosed
    by an increase in pulmonary arterial blood pressure that is driven by a progressive vascular
    remodelling of small pulmonary arterioles. We have previously reported that tumor
    necrosis factor apoptosis-inducing ligand (TRAIL) protein expression is increased in
    pulmonary vascular lesions and pulmonary artery smooth muscle cells (PASMC) of
    patients with idiopathic PAH. The addition of recombinant TRAIL induces the
    proliferation and migration of PASMCs in vitro. TRAIL is required for hypoxia-induced
    pulmonary hypertension in mice, and blockade of TRAIL prevents and reduces disease
    development in other rodent models of PAH.
  • August 9, 2014
  • Arrhythmogenesis in Pulmonary Hypertension
  • (University of Manchester Library) Pulmonary arterial hypertension (PAH) is a condition with severe morbidity and mortality. It is associated with an increase in incidence of all forms of arrhythmias which further increase morbidity and mortality. The monocrotaline (MCT) model of pulmonary hypertension (PH) in the rat is analogous to PAH in humans and was used to study how PH causes arrhythmias.
  • August 8, 2014
  • 'Stem cells show promise in stroke recovery'
  • (BBC) Although not directly PAH related, it is an indication of progressing Stem Cell research that will hopefully lead on day to better PH treatments.
    Infusing stem cells into the brain may help boost recovery after a stroke, according to a pilot study by Imperial College London.
    Scientists believe the cells encourage new blood vessels to grow in damaged areas of the brain.
    They found most patients were able to walk and look after themselves independently by the end of the trial, despite having suffered severe strokes.
  • August 7, 2014
  • Discovery sheds light on how changes in lungs can hurt the heart
  • (MedicalXpress) A team of UW-Madison researchers has discovered important biomechanical changes in human arteries that could increase understanding of how pulmonary hypertension leads to heart failure.
  • August 6, 2014
  • Macitentan Effective in Treating PAH During Unique Trial
  • (PHNews) Endothelin-receptor antagonists (ERAs), recently discussed on Pulmonary Hypertension News as being effective for treating pulmonary hypertension, have been a standard part of clinician’s portfolios along with phosphodiesterase type 5 inhibitors and calcium channel blockers
  • August 5, 2014
  • Emphysema Burdens PH Patients
  • (PHNews) Pulmonary hypertension (PH) on its own is burdensome to the patients it afflicts, but when PH is combined with other diseases, the conditions can be devastating.
  • August 4, 2014
  • Early detection of chronic thromboembolic pulmonary hypertension
  • (PH News) A study presented at the Annual Congress of the European Respiratory Society in Barcelona, Spain, identified the benefits of a telephone monitoring program in helping to diagnose CTEPH in its early stages following a diagnosis of pulmonary embolism.
  • August 3, 2014
  • 'I Feel Like I'm Suffocating' -- Defining the Experience of Breathlessness
  • (Medscape) Episodic breathlessness is a common and distressing symptom in patients with advanced disease. However, breathlessness is not yet clearly defined. The aim of this project was to develop an international definition, categorization, and terminology of episodic breathlessness.
  • Exciting IPF Treatment News Must Be Interpreted Cautiously
  • (Medscape) In this video clip, Andrew F. Shorr, MD, MPH, from Washington, DC, discusses the 3 articles about idiopathic pulmonary fibrosis (IPF) that were all  published in the May 29 issue of the New England Journal of Medicine.
  • August 2, 2014
  • African Americans Tend to Have More Severe Systemic Sclerosis-Associated PAH
  • (Pulmonary Hypertension News) More often than not, risk factors for and development rates of cardiovascular diseases vary according to race and ethnicity, and pulmonary arterial hypertension due to systemic sclerosis is no exception. A study from Johns Hopkins University, appearing in the journal European Respiratory Journal, identified the fact that African Americans tend to have more severe systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH).
  • Pulmonologist Recommends PAH Patients Consult Doctors Before High Altitude Exposure
  • (Pulmonary Hypertension News) A recent blog article entitled, “How High Is too High – Altitude and PAH” from pulmonology specialist Jeremy P. Feldman, published on the blog Pulmonary Hypertension R.N. is encouraging patients with pulmonary arterial hypertension to consult their physicians prior to airplane travel and exposure to high altitudes, noting that physicians can help PAH patients make the right decisions on a case by case basis.
  • August 1, 2014
  • Macitentan Effective in Treating PAH
  • (Pulmonary Hypertension News) As new medications for pulmonary hypertension are approved and marketed, it is important to evaluate their efficacy, both absolutely and relative to tried-and-true products. Macitentan, marketed under the name Opsumit by Actelion, is approved to treat pulmonary arterial hypertension patients under the World Health Organization classification of Group 1 in the United States and Functional Classes 2 and 3 in the European Union. It was recently reviewed in an article published in Drugs and found to be a useful treatment option with the added benefit of once-daily administration.
  • July 31, 2014
  • Rubella-Related PH Leads to Infant Mortalities
  • (Pulmonary Hypertension News) Pulmonary hypertension can be a devastating disease, especially when it impacts infants and children. A new study in Pediatrics from Nagasaki University and Nagasaki University Hospital in Japan and Khanh Hoa General Hospital and Health Service in Vietnam found high rates of mortality from pulmonary hypertension in infants with congenital rubella syndrome..
  • July 30, 2014
  •  Drugs used to treat lung disease work with the body clock
  • (Medical Xpress) In the UK pneumonia, which is caused by an infection, affects around 1 in 1000 adults each year and is more serious for babies, young children, the elderly, smokers and those with an underlying health condition
  • Portopulmonary hypertension: Improved detection using CT and echocardiography in combination.
  • ( CT contributes to the non-invasive detection of portopulmonary hypertension when used in a diagnostic algorithm with transthoracic echocardiography. CT may have a role in the pre-liver transplantation triage of patients with portopulmonary hypertension for right-sided heart catheterisation.
  • July 29, 2014
  • Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design?
  • ( Idiopathic pulmonary fibrosis (IPF) is a disease that carries a high mortality. Pulmonary hypertension (PH) frequently complicates the course of patients with IPF and is associated with significantly worse outcomes. Whether PH is a surrogate or driver of these worse outcomes remains unanswered, but the presence of PH represents an attractive target for therapy. This review delves into the various pulmonary vasoactive agents that have been subjected to study in IPF, the pitfalls of some of these prior studies, and attempts to lay a foundation for future study designs targeting PH in IPF...(downloadable full free text paper)
  • July 27, 2014
  • The Potential for Macitentan, a New Dual Endothelin Receptor Antagonist, in the Treatment of Pulmonary Arterial Hypertension
  • (Medscape) In recent years in the management of pulmonary arterial hypertension (PAH), endothelin receptor antagonists (ERAs) represent a well-established class of therapeutic agents with clear beneficial effects. Macitentan (Opsumit®), a dual ERA optimized for efficacy and safety, is the newest drug in the class. Macitentan presents a number of key beneficial characteristics, including increased in vivo preclinical efficacy versus existing ERAs, resulting from sustained receptor binding and physicochemical properties that allow enhanced tissue penetration. The clinical pharmacokinetics studies also indicated a low predilection of macitentan for drug–drug interactions. In the SERAPHIN trial, a phase III long-term study of PAH, macitentan significantly reduced morbidity and mortality by 45% versus placebo, providing sustained long-term improvements in exercise capacity. No association was found between changes in exercise capacity and long-term clinical outcomes, but improved cardiopulmonary hemodynamics were recorded in macitentan-treated patients irrespective of baseline background PAH therapy or World Health Organization functional class. Based on these favorable data, the US Food and Drug Administration approved the 10 mg/day dose in late 2013 and the same process has recently been concluded by the European Medicines Agency.
  • Pulmonary Hypertension Is Related to Peripheral Endothelial Dysfunction in Heart Failure With Preserved Ejection Fraction
  • (AHA Journals) Pulmonary hypertension and collagen metabolism abnormalities are prevalent in patients with HFpEF. Peripheral endothelial dysfunction (PED) has been described in heart failure (HF), as well as in pulmonary arterial hypertension. Our aim is to determine whether pulmonary hypertension is associated with PED and impaired collagen metabolism in patients with heart failure and preserved ejection fraction (HFpEF).
  • Specialty drugs transform lives — but at a cost
  • (Boston Globe) Expensive new specialty drugs offer hope to patients with rare and chronic illnesses but also leave many worrying about paying for them.
  • July 26, 2014
  • Mustaches May Raise Burn Risk With Home Oxygen Therapy
  • (Medscape) A new case series suggests that men on home oxygen therapy should consider a clean-shaven look to reduce their risk of serious facial burns.
  • Up-Regulated LCN2 in PH Involving in PASMC Resistance to Apoptosis
  • (Int J Biol Sci) A key feature of pulmonary hypertension (PH) is the remodeling of small pulmonary arteries due to abnormal pulmonary artery smooth muscle cell (PASMC) proliferation and resistance to apoptosis. However, the cellular mechanisms underlying how PASMCs in the pathological condition of pulmonary hypertension become resistant to apoptosis remain unknown. It was recently reported that lipocalin 2 (Lcn2) is up-regulated in a wide array of malignant conditions, which facilitates tumorigenesis partly by inhibiting cell apoptosis. In this study, we observed that the expression levels of Lcn2 were significantly elevated in a rat PH model induced with monocrotaline and in patients with congenital heart disease-associated PH (CHD-PH) when compared with respective control.
  • July 25, 2014
  • Making Pulmonary Pressure Measurements Safer For PH Patients Using Ultrasound
  • (Pulmonary Hypertension News) A group of researchers from Flinders Medical Centre and Flinders University in Australia are working to make measuring pulmonary pressures safer for patients with pulmonary hypertension. Their study, published in Critical Ultrasound Journal,investigated the use of ultrasound-echocardiography for estimating mean pulmonary arterial pressure in critically ill patients. Although the results were not on-par with those from the accepted standard, more work may refine the technique to provide a less invasive monitoring system.
  • July 24, 2014
  • Major Signaling Pathway in Scleroderma May Enable Effective Treatments, Cures
  • (Pulmonary Hypertension News) As any scleroderma patient knows, the disease has no cure and no effective treatments exist. The main goal of current treatments is reducing inflammation which, often times, is not enough to provide relief to patients, especially those who have developed pulmonary hypertension as a result of thickened pulmonary arteries.
  • July 23, 2014
  • Scleroderma Patients Benefit from Stem Cell Transplants Over Conventional Treatment
  • (Pulmonary Hypertension News) Scleroderma patients, who are sometimes affected by pulmonary hypertension, are becoming routinely treated with stem cell transplants. Dr. Dinesh Khanna, at the annual European Congress of Rheumatology, previewed the results of the Autologous Stem Cell Transplantation International Scerloderma (ASTIS) trial evaluating 156 severe scleroderma patients randomized to receive a stem cell transplant or cyclophosphamide, a conventional drug used to treat some auto-immune diseases because it decreases the immune system’s response.
  • July 22, 2014
  • New PAH-Related Hospitalization Study Released
  • (Pulmonary Hypertension News) Lead author Charles Burger, MD, from the Mayo Clinic, and colleagues reported their findings in Chest. Data was gathered for 862 patients, and 490 (56.8%) experienced at least one hospitalization during their first three years post-diagnosis. 490 of the total had experienced at least one hospitalization during the 3-year follow-up period. 52.4% of the hospital admissions were PAH-related, while 43.7% were unrelated to the disease and 3.9% were due to undetermined causes. Half were related to the patients’ PAH, and the mean total number of days spent in the hospital was 15.3 days. Not only is a hospital stay burdensome to patients, but also it places a burden on the healthcare system.

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