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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.



  • July 29, 2015
  • World’s First Clinical Trial Of Genetically-enhanced Stem Cell PAH Therapy Yields Promising Findings
  • (Lung Disease News) A team of Canadian researchers have published promising results of the first clinical trial in the world of a genetically enhanced stem cell therapy for pulmonary arterial hypertension (PAH).
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  • Bellerophon Therapeutics Expands License Agreement With INO Therapeutics to Develop INOpulse(R) for Three Additional Cardiopulmonary Diseases
  • (Nasdaq) Bellerophon Therapeutics, Inc. (Nasdaq:BLPH), a clinical-stage biotherapeutics company, today announced that it has expanded its license agreement with INO Therapeutics LLC, a division of Mallinckrodt plc (NYSE:MNK), allowing Bellerophon to develop INOpulse® for the treatment of three additional cardiopulmonary diseases, including chronic thromboembolic pulmonary hypertension (CTEPH), pulmonary hypertension associated with ...
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  • July 28, 2015
  • Taking aim at pulmonary hypertension in heart failure with preserved ejection fraction
  • (European Heart Journal) About half of people with heart failure (HF) have a preserved ejection fraction (HFpEF). In contrast to HF with reduced EF (HFrEF), randomized controlled trials in HFpEF have failed to identify an effective treatment to date, and clinicians continue to struggle to manage this growing cohort of patients.  While previously believed to be caused exclusively by diastolic dysfunction, the pathophysiology of HFpEF is now recognized to be far more complex.
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  • July 27, 2015
  • FDA Issues Pulmonary Hypertension Warning For Babies Treated With Diazoxide
  • (American College of Cardiology) The U.S. Food and Drug Administration (FDA) on July 16 issued a warning for Proglycem (diazoxide) after reports of pulmonary hypertension in infants and newborns treated with the drug for low blood sugar. The FDA reports that the pulmonary hypertension improved after Proglycem was stopped. The agency urges health care professionals to closely monitor infants and newborns being treated with the drug and stop treatment if pulmonary hypertension occurs.
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  • July 26, 2015
  • OFEV (nintedanib) Added To Updated International Treatment Guidelines for Idiopathic Pulmonary Fibrosis (IPF)
  • (Pulmonary Hypertension News) Revised international treatment guidelines for Idiopathic Pulmonary Fibrosis (IPF) suggest use of OFEV (nintedanib), a proprietary drug from Ingelheim, Germany, based Boehringer Ingelheim Corporation for the treatment of the disease. This newly-added recommendation places high value on potential benefits of OFEV on patient-important outcomes such as slowing of disease progression measured by the rate of forced vital capacity (FVC) — the amount of air which can be forcibly exhaled from the lungs after taking the deepest breath possible — decline, and takes into account potential significant adverse effects and the expected cost of treatment.
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  • MUSC and Bristol-Myers Squibb Partner for Fibrotic Disease Research
  • (Pulmonary Hypertension News) Bristol-Myers Squibb Company and the Medical University of South Carolina recently signed a translational research partnership that will focus on fibrotic diseases, such as scleroderma, renal fibrosis, and idiopathic pulmonary fibrosis. This collaboration will also cover studies developed to further explore the disease mechanism of fibrosis, patient segmentation, and their biomarkers or predictors.
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  • July 25, 2015
  • Promising Genetically-Enhanced Stem Cell Therapy for Pulmonary Arterial Hypertension Discovered
  • (Pulmonary Hypertension News) A new study recently published in the journal Circulation Research revealed encouraging results in the world’s first clinical trial using a genetically-enhanced stem cell therapy for pulmonary arterial hypertension (PAH). The study is entitled “Endothelial NO-Synthase Gene-Enhanced Progenitor Cell Therapy for Pulmonary Arterial Hypertension: the PHACeT Trial,” and was led by researchers at the University Health Network and the Ottawa Hospital Research Institute in Canada.
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  • Hemodynamic Characteristics Including Pulmonary Hypertension at Rest and During Exercise Before and After Heart Transplantation
  • (Journal of the American Heart Association) Little is known about the hemodynamic response to exercise in heart failure patients at various ages before and after heart transplantation (HT). This information is important because postoperative hemodynamics may be a predictor of survival. To investigate the hemodynamic response to HT and exercise, we grouped our patients based on preoperative age and examined their hemodynamics at rest and during exercise before and after HT.
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  • July 24, 2015
  • Pulmonary Hypertension in the setting of Hereditary Hemorrhagic Telangiectasia.
  • (PHA) PH in patients with HHT occurs by different mechanisms, and there is a trend towards worse survival in patients that develop PH despite the mechanism. The equal predilection towards all subtypes of PH illustrates the necessity of RHC to clarify the hemodynamics.
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  • Early markers of right heart involvement in regular smokers by Pocket Size Imaging Device.
  • PSID = Pocket Size Imaging Device detects early ultrasound signs of RH involvement in regular otherwise healthy smokers in comparison with PE.
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  • July 23, 2015
  • 6 min walk resaults predictive für PAH in connective tissue disease
  • (Medical Express) The 6MW stress echocardiography noninvasively provides an incremental prognostic value of PH development in CTD," the authors write. "Larger multicenter studies are warranted to confirm the result."
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  • July 22, 2015
  • Pulmonary Hypertension Pioneer Dr. Stuart Rich Joins Northwestern Medicine
  • (PR Newswire) CHICAGO, July 22, 2015 /PRNewswire-USNewswire/ -- Northwestern Medicine welcomes Stuart Rich, MD, as director of the new Pulmonary Vascular Disease Program at the Bluhm Cardiovascular Institute, and as a professor of medicine-cardiology at Northwestern University Feinberg School of Medicine. Rich is a pioneer in the treatment of pulmonary hypertension, an incurable and debilitating type of high blood pressure, and widely considered one of the world's most recognized experts on the disease.

    "I'm a dreamer, and the leadership in the Bluhm Cardiovascular Institute is strong and visionary," said Rich. "Northwestern Medicine is the ideal setting for the next big breakthrough in the treatment of pulmonary hypertension, and I look forward to working with the strong team in place to further advance treatment for this chronic disease."
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  • Sildenafil Found Ineffective in Diastolic Left Heart Failure Patients with PH
  • (Pulmonary Hypertension News) A new study from University Medical Center Gronigen in The Netherlands, released on fast-track by the European Heart Journal, reports that a clinical trial treating pulmonary hypertension patients with sildenafil did not result in reduced pulmonary artery pressure (PAP) or haemodynamic parameters. These patients had preserved ejection fraction (diastolic heart failure) with their pulmonary hypertension, meaning the amount of blood pumped out of the left ...
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  • July 21, 2015
  • Endothelial NO-Synthase Gene-Enhanced Progenitor Cell Therapy for Pulmonary Arterial Hypertension: the PHACeT Trial
  • (AHAJournals) Delivery of EPCs overexpressing eNOS was tolerated hemodynamically in patients with PAH. Furthermore, there was evidence of short-term hemodynamic improvement, associated with long-term benefits in functional and QOL assessments. However, future studies are needed to further establish the efficacy of this therapy. This trial was initated in 2006, almost a decade ago, finally published, now. 
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  • The Causal Role of IL-4 and IL-13 in Schistosoma mansoni Pulmonary Hypertension.
  • (Pubmed) Combined IL-4 and IL-13 deficiency is required for protection against TGF-β-induced pulmonary vascular disease after Schistosoma exposure, and targeted inhibition of this pathway is a potential novel therapeutic approach for patients with schistosomiasis-associated PAH.

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  • July 20, 2015
  • Two seismic shifts in Medicare, and what they mean for you
  • (PBS.org) Did you feel the ground shift beneath your feet last week? Let me rephrase that to include West Coast readers: Did you feel the ground shift a lot beneath your feet last week? Well, it did, at least in terms of Medicare benefits and how the agency goes about its core job of paying health care providers for the care they provide to Medicare beneficiaries.
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  • Bronchoscopic Lung Volume Reduction Benefits Subset of Emphysema Patients
  • (Medscape) Bronchoscopic lung volume reduction (BLVR) with endobronchial valves is especially effective for emphysema patients with intact interlobar fissures, according to results from the BeLieVeR-HIFi study.
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  • July 19, 2015
  • Comparing New Treatments for Idiopathic Pulmonary Fibrosis
  • (Medscape) The treatment landscape for idiopathic pulmonary fibrosis, a devastating lung disease, is changing. To investigate the effectiveness of treatments for idiopathic pulmonary fibrosis we undertook a systematic review, network meta-analysis and indirect comparison.
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  • Lixiana (Edoxaban) for DVT and PE Receives NICE Recommendation in UK
  • (Pulmonary Hypertension News) UK Patients at risk of developing venous thromboembolism (VTE), which includes thrombi and embolisms in the legs (deep vein thrombosis) and lungs (pulmonary embolism), may soon have the opportunity to gain access to a new treatment for the condition. The National Institute for Health and Care Excellence (NICE) recently issued a Final Appraisal Determination (FAD) for Lixiana (edoxaban), an oral, once-daily, direct factor Xa (pronounced “Ten A”) inhibitor, which works to help thin the blood and address conditions such as PE.
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  • July 18, 2015
  • New Sound Wave-Based Method for Assessing Heart Function in PAH Shows Promise in New Study
  • (Pulmonary Hypertension News) A new imaging method that uses sound waves could help in assessing heart damage in people with pulmonary arterial hypertension (PAH), according to a recent study that appeared June 26 in the Journal of Cardiovascular Ultrasound.
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  • July 17, 2015
  • Effects of the Pulmonary Hypertension Manual (PulHMan) on Awareness of Exercise in Patients with Pulmonary Hypertension.
  • (PubMed) Patient education is an important part of holistic care for patients with chronic diseases. With the success of the Heart Manual, it was felt there is a strong need to develop such a manual for those with pulmonary hypertension (PH) - the Pulmonary Hypertension Manual (PulHMan). This was developed through a qualitative methodology in order to ensure the needs of the patients were being met. The impact of the PulHMan on awareness about exercise-related benefits and PH are not known. Therefore the aim of this study was to assess the effects of the PulHMan on improving awareness among individuals with PH.
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  • CT and MR imaging measurements reflect symptoms of progressive lung disease
  • (2 Minute Medicine) In patients with mild-to-moderate chronic obstructive pulmonary disease (COPD), pulmonary MRI and CT imaging identified disease phenotypes, exercise limitations and predicted symptomology, particularly in patients with minimal FEV1 abnormalities.
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  • July 16, 2015
  • Arginine Therapy May Alleviate PH in Thalassemia Patients
  • (Pulmonary Hypertension News) The amino acid arginine may contribute to pulmonary hypertension in patients with thalassemia, a disorder that leads to abnormal oxygenation of blood. A team of researchers from the Thalassemia Clinical Research Network conducted a study demonstrating that abnormal release of the enzyme arginase leads to a lack of bioavailable arginine, leading to downstream effects on blood pressure. After studying 27 patients with thalassemia, the researchers now have a clearer picture of why pulmonary hypertension can be present in these patients.
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  • Researchers Uncover A New and Deeper Understanding of Lung Regeneration Pathways
  • (Lung Disease News) In a new study entitled “Time? and compartment?resolved proteome profiling of the extracellular niche in lung injury and repair,” researchers uncovered the dynamic response of lungs to injury and which pathways are activated for repair. The study was recently published in the EMBO Molecular Systems Biology journal.
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  • Determinants of an elevated pulmonary arterial pressure in patients with pulmonary arterial hypertension
  • (7th Space) Given the difficulty of diagnosing early-stage pulmonary arterial hypertension (PAH) due to the lack of signs and symptoms, and the risk of an open lung biopsy, the precise pathological features of presymptomatic stage lung tissue remain unknown. It has been suggested that the maximum elevation of the mean pulmonary arterial pressure (P pa ) is achieved during the early symptomatic stage, indicating that the elevation of the mean P pa is primarily driven by the pulmonary vascular tone and/or some degree of pulmonary vascular remodeling completed during this stage.
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  • July 15, 2015
  • 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.
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  • Breath of life
  • (Orange County Register) Placentia’s Julie Hancock feels like she’s won the lottery − twice.

    Hancock received the first lung transplant ever performed at UCLA Medical Center 25 years ago. She had had primary pulmonary hypertension, a rare lung disease that leads to narrowing of the blood vessels of the lungs.
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  • Fatal Pulmonary Embolism Case Report Highlights Need For More Attention To Factitious Fever Risks
  • (Lung Disease News) In a recent study entitled “Fatal non-thrombotic pulmonary embolization in a patient with undiagnosed factitious disorder,” the authors present a case study of a patient with undiagnosed factitious fever that resulted in the patient’s death and highlight the urgent need for early recognition of these disorders so that prevention measures can be introduced before they become fatal. The study was published in the BMC Research Notes journal.
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  • July 14, 2015
  • Roche’s ESBRIET (pirfenidone) Deemed Safe in Systemic Sclerosis Patients with Interstitial Lung Disease
  • (Pulmonary Hypertension News) Results showing the safety of Esbriet (pirfenidone) in treating interstitial lung disease due to systemic sclerosis were recently presented at the European League Against Rheumatism Annual European Congress of Rheumatology. The U.S. Food and Drug Administration approved Esbriet (pirfenidone), which is currently marketed by Roche, as a treatment for idiopathic pulmonary fibrosis (IPF) in the Fall of 2014. Now, researchers have evaluated the therapy’s safety in patients with interstitial lung disease, which can arise as a complication of systemic sclerosis.
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  • SteadyMed and Cardiome Partner To Commercialize PAH Therapy Trevyent Outside The U.S.
  • (Pulmonary Hypertension News) SteadyMed Ltd., a pharmaceutical company developing therapies for both high value and orphan diseases with unmet parenteral delivery needs, and Cardiome Pharma Corp. have recently announced an exclusive agreement for Cardiome to market Trevyent in specific markets outside the United States if Trevyent receives approval to treat pulmonary arterial hypertension (PAH) in those regions. The license agreement stipulates that SteadyMed will receive $12.25 million linked to regulatory and sales milestones, and will include a $3 million upfront payment. In addition, Cardiome will pay royalties on future sales of Trevyent to SteadyMed. This exclusive partnership includes Canada, Europe and the Middle East.
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  • July 13, 2015
  • Novel Technique Promises a Lung Sealant for Pulmonary Emphysema
  • (Medscape) Emphysema is a component of chronic obstructive pulmonary disease (COPD) that is found, along with chronic bronchitis, in most patients with COPD. Patients with predominant emphysema tend to be very symptomatic, with shortness of breath, even on mild exertion, being their worst problem.
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  • July 12, 2015
  • Pulmonary Embolism and Pulmonary Hypertension
  • (Pulmonary Hypertension RN) The majority of patients with pulmonary embolism can be managed with blood thinners such as Heparin, Lovenox, Xarelto, Eliquis or Warfarin.  Over time, blood thinners prevent further blood clots and allow your body to dissolve the existing blood clot.  More than 95% of patients will do very well with blood thinners and their symptoms should resolve within a few weeks to a couple of months.  By three months, the majority of patients have had maximal resolution of their blood clots.
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  • Fixed Low-dose Ultrasound-assisted Catheter-directed Thrombolysis for Intermediate and High-risk Pulmonary Embolism
  • (Medscape) A standardized catheter intervention approach using fixed low-dose USAT for the treatment of intermediate- and high-risk PE was associated with rapid improvement in haemodynamic parameters and low rates of bleeding complications and mortality.
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  • Warfarin: Double-Edged Sword After PE
  • (MedPage Today) After a first-time, unprovoked pulmonary embolism, an additional 18 months of warfarin -- beyond the initial 6-month therapy -- reduced the risk of a major event compared with placebo, though the risk of major bleeding persisted, researchers found.
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  • July 11, 2015
  • PAH and Diuretics
  • (Pulmonary Hypertension RN) One of the major problems in PAH is that patients retain fluid. That means that all of the salt and fluids you drink are not adequately eliminated by your kidneys. Normally the body has a meticulous balance between salt and water in and urine out. With PAH, the heart sends hormonal signals to the kidneys to retain salt and water. The net effect is that over time salt and water accumulate and edema or swelling in the legs and abdomen develops. Swelling in ankles of PH patientUnfortunately as this process gets worse the heart has a harder time pumping blood and there is an even greater signal sent to the kidneys to retain fluid and salt. This feed forward cycle leads to symptomatic right heart failure. When you visit your doctor and the doctor checks your ankles, pushes on your stomach and inspects your neck veins, we are looking for signs of fluid retention.
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  • Prognosis still gloomy for idiopathic PAH
  • (News Medical) Analysis of the REVEAL registry shows poor 5-year outcomes for patients with pulmonary arterial hypertension (PAH), despite improvements due to targeted therapies.
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  • July 9, 2015
  • Novel Portable Nitric Oxide Generator for Pulmonary Hypertension Therapy
  • (Med Gadget) Dr. Warren Zapol’s pioneering studies two decades ago led to the implementation of inhaled nitric oxide (NO) for the treatment of pulmonary hypertension in infants, children, and adults. An effective vasodilator, inhaled NO relaxes pulmonary blood vessels without causing a systemic drop in blood pressure, and is considered a life-saving therapy, particularly in infants and children who suffer from hypoxia and respiratory failure. Despite the efficacy of inhaled NO treatment, factors such as high cost and bulky, complex equipment limit its widespread availability
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  • July 8, 2015
  • Team generates therapeutic nitric oxide from air with an electric spark
  • (MedicalSpress) "Nitric oxide is used to treat about 35,000 hospitalized U.S. patients each year - mostly adults with pulmonary hypertension and infants with a condition called persistent pulmonary hypertension of the newborn (PPHN)," says Warren M. Zapol, MD, director of the MGH.
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  • July 7, 2015
  • Researchers Advance Pulmonary Embolism Diagnosis Through Computed Tomography
  • (Pulmonary Hypertension News) Researchers at Leiden University Medical Center in The Netherlands recently published in the journal Diagnostic and Interventional Radiology a review on the value of computed tomography (CT) as a diagnostic tool for pulmonary embolism. The study is entitled “The role of computed tomography in the diagnosis of acute and chronic pulmonary embolism.”
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  • July 6, 2015
  • Admission Rates Rise for Pulmonary Hypertension in Kids
  • (MedPage Today) While pediatric hospitalizations and costs associated with pulmonary hypertension (PH) have increased, mortality has declined, according to a large retrospective cohort study of hospital data from 1997 to 2012.
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  • July 5, 2015
  • Autoantibodies Targeted in New Strategy to Treat IPF
  • (Pulmonary Hypertension News) Novel research investigating the relationship between autoantibodies and idiopathic pulmonary fibrosis (IPF) may allow new therapies to be developed based on current treatments for autoimmune diseases. The laboratory of Steve Duncan, MD, at the University of Alabama at Birmingham Division of Pulmonary, Allergy, and Critical Care Medicine recently published a study in PLOS One that described how autoantibodies play an important role in IPF when patients are undergoing acute exacerbations.
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  • Stress-Doppler-Echocardiography for early detection of systemic sclerosis associated pulmonary arterial hypertension
  • (7th Space) The results of this prospective, cross-sectional study using RHC as gold standard in all patients showed that SDE markedly improved sensitivity in detecting manifest PH to 95.2% compared to 72.7% using echocardiography at rest only. Thus, for PH-screening in SSc-patients echocardiography should be performed at rest and during exercise.
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  • July 4, 2015
  • Pulmonary Hypertension - Education Videos
  • (Khan Academy) In it's mission to provide a free, world-class education for anyone, anywhere, the Khan Academy offers the following six videos in it's Pulmonary Hypertension educational series:  "What is pulmonary hypertension?", "Pulmonary hypertension symptoms and causes", "Hypoxic vasoconstriction", "Pulmonary hypertension diagnosis Swan Ganz catheterization", "Pulmonary hypertension diagnosis" and "Pulmonary hypertension treatment".
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  • July 3, 2015
  • CTEPH Patients Can Develop Coronary-Pulmonary Collateral Vessels, According To Study
  • (Pulmonry Hypertension News) Researchers at University of California, San Diego (UCSD) recently published findings in the journal Pulmonary Circulation revealing the prevalence of specific collateral arteries in patients with chronic thromboembolic pulmonary hypertension (CTEPH). The study is entitled “Prevalence of coronary artery-pulmonary artery collaterals in patients with chronic thromboembolic pulmonary hypertension.”
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  • July 2, 2015
  • Blood Clots: the Least-Appreciated Complication of Hospital Care?
  • (US News Health) If you were undergoing a surgical procedure, would you ever think to refuse the antibiotics your physician had ordered to prevent an infection?
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  • Producing nitric oxide by pulsed electrical discharge in air for portable inhalation therapy
  • (Science Magazine) Inhalation of nitric oxide (NO) produces selective pulmonary vasodilation and is an effective therapy for treating pulmonary hypertension in adults and children.
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  • June 30, 2015
  • Uric Acid Levels in SLE Patients Associated With PH in New Study
  • (Pulmonary Hypertension News) Uric acid is most often associated with gout in patients. However, a new study published in International Journal of Rheumatic Diseases found that patients with systemic lupus erythematosus (SLE) may be at an increased risk for pulmonary hypertension based on high uric acid levels.
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