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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.



  • September 23, 2016
  • A case of postpartum pulmonary hypertension complicated with mixed connective tissue disease
  • (J-Stage) A 34-year-old pregnant woman was diagnosed with mixed connective tissue disease (MCTD), complicated with postpartum pulmonary hypertension (PH). She was taking 10 mg oral prednisolone during pregnancy. At 29 weeks’ gestation, systolic blood pressure increased and proteinuria developed, and she was admitted for preeclampsia. However, at 32 and 3/7 weeks’ gestation, her blood pressure elevated to 195/117 mmHg, and her platelet count decreased to 96×103/μl. Thus, she was diagnosed as severe preeclampsia, and an emergency cesarean section was performed. At postpartum 1 day, she complained of dyspnea and palpitation. Pericardial effusion and right ventricle enlargement were detected on cardiac ultrasound. Her tricuspid regurgitation peak gradient was elevated (40 mmHg), indicating the presence of PH. Accordingly, prednisolone 30 mg/day was administered, and her dyspnea improved. Her blood pressure decreased and her proteinuria disappeared on day 10 postpartum. She was discharged on day 14 with oral prednisolone. Evaluation of cardiac and pulmonary functions before pregnancy in MCTD-complicated patients and careful observation for PH during and after pregnancy is recommended.
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  • Creating 3-D lungs in the lab may lead to better treatment for lung disease
  • (RawStory) Using stem cells, researchers at UCLA have successfully grown three-dimensional human lung “organoids.” The study may lead to improved treatment for lungs scarred by disease.
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  • September 22, 2016
  • Diabetes and Obesity Treatment Shows Promise in Preventing in Early Study
  • (Pulmonary Hypertension News) Liraglutide, a drug used to treat type 2 diabetes (brand name, Victoza) and obesity (brand name, Saxenda), is currently being evaluated as a possible therapy for Parkinson’s disease. Now, a new study suggests that liraglutide may also be quite effective in treating pulmonary arterial hypertension (PAH).
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  • Anastrozole in Pulmonary Arterial Hypertension (AIPH): A Randomized, Double-Blind Placebo-Controlled Trial
  • (ATS Journals) Anastrozole significantly reduced E2 levels in patients with PAH, but had no effect on TAPSE. Anastrozole was safe, well-tolerated, and improved 6MWD in this small “proof-of-principle” study. Larger and longer Phase II clinical trials of anastrozole may be warranted in patients with PAH.
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  • September 21, 2016
  • Familial Chronic Thromboembolic Pulmonary Hypertension in a Pair of Japanese Brothers
  • (Chest) The cases of CTEPH as described by Desmarais and Elliott and the two brothers in the current report provide evidence that familial CTEPH exists, although it seems to be rare. In Japan, at least, the downward trend in the birth rate may mask the actual prevalence of familial CTEPH. By undertaking a worldwide collaboration to study more families with CTEPH, we can expect to elucidate the possible molecular mechanisms involved in the pathogenesis of CTEPH, including currently unknown genetic factors.
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  • Imatinib could be a new strategy for pulmonary hypertension caused by pulmonary tumor thrombotic microangiopathy in metastatic breast cancer
  • (7th Space) IntroductionPulmonary tumor thrombotic microangiopathy (PTTM) is rare, cancer-related pulmonary complication leading to hypoxia, pulmonary hypertension, and heart failure. The standard treatment for PTTM is not established.
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  • September 20, 2016
  • Genetically modified probiotic may treat pulmonary hypertension
  • (UPI.com) Using a probiotic bacteria, researchers delivered a peptide to rats' hearts that reduced symptoms of the chronic condition.
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  • New Paradigm for Pulmonary Arterial Hypertension Treatment
  • (Medscape) Pulmonary arterial hypertension (PAH) was previously considered a uniformly fatal disease, with patients succumbing to right heart failure and death at an average of 3 years after diagnosis. The past 20 years, however, have seen the development of numerous targeted therapies that have changed the natural history of PAH. As more pharmacologic agents have been approved and utilized, further advances in the design of and endpoints for clinical trials. This study will review some of these notable developments.
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  • September 19, 2016
  • Treprostinil Long-term IV Infusion for PAH: A New Device for Treating Pulmonary Arterial Hypertension
  • (Medscape) Pulmonary arterial hypertension (PAH) is a serious disorder with a mortality rate of 10%-15% annually. The three well-studied classes of drugs for PAH (prostanoids, endothelial receptor antagonists, and phosphodiesterase-5 [PDE-5] inhibitors) all have disadvantages, and none offers the convenience of use necessary for satisfactory therapy. A prostacyclin drug is generally considered the most effective agent, but the method of administration—frequent intravenous (IV) infusions—has been a disadvantage until now. The recent development of a fully implantable IV delivery system offers more convenience because the device is programmable and needs to be refilled only every 12 weeks. The results of a clinical trial of this device have just been made available.
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  • September 16, 2016
  • Efficiency of Mifcare’s Anti-inflammatory Compound for PAH Shown in Second Animal Model
  • (Pulmonary Hypertension News) The French biotechnology company Mifcare recently announced that their lead anti-inflammatory compound MFC1040 has been shown to be efficient in a second animal model of pulmonary arterial hypertension (PAH).
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  • New technology helps to protect lungs before transplants
  • (10 TV) An estimated 35 million people in the U.S. have chronic lung disease but the number of lung transplants pales in comparison.
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  • September 15, 2016
  • Bronchoscopy Safe for Patients with PH
  • (Pulmonary Hypertension News) A new study, published in the scientific journal Respiration, suggests that these techniques are, in fact, safe in patients with echocardiographic evidence of PH.

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  • September 14, 2016
  • Mixed connective tissue disease presenting with isolated pulmonary hypertension, and limited cutaneous sclerosis
  • (International Journal of Research in Medical Sciences) Mixed connective tissue disease (MCTD) was first recognized by Sharp and Colleagues in 1972 among a group of patients with overlapping clinical features of systemic lupus erythematosis (SLE), scleroderma and myositis, with the presence of distinctive antibodies against, what now is known to be U1ribonucleoprotein (RNP). We report an unusual case of a 29-year old female with MCTD characterized by the coexistence of signs, symptoms and immunological features of 3 defined autoimmune diseases isolated PHT, cutaneous sclerosis (SSc), hypothyroidism
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  • Molecular Pathway, Newly Linked to PAH, May Work to Protect Lungs
  • (Pulmonary Hypertension News) Both patients newly diagnosed with pulmonary arterial hypertension (PAH) and animal models of the disease show increased activity of a molecular pathway, known as NNMT-MNA (Nicotinamide N-methyltransferase and 1-methylnicotinamide), which is involved in metabolic and inflammatory reactions.
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  • September 13, 2016
  • Single Lung Transplant in Moderate to Severe Pulmonary Hypertension
  • (4th Global Surgery and Transplantation Congress) Advanced lung disease (ALD) that requires lung transplantation (LTX) is frequently associated with pulmonary hypertension (PH). Whether the presence of PH significantly affects the outcomes following single-lung transplantation (SLT) remains controversial. Therefore, we retrospectively examined the outcomes of 279 consecutive SLT recipients transplanted at our center, and the patients were split into four groups based on their mean pulmonary artery pressure values. Outcomes, including long-term survival and primary graft dysfunction, did not differ significantly for patients with versus without PH, even when PH was severe. We suggest that SLT can be performed safely in patients with ALD-associated PH thereby increasing the impact of donors on the available organ pool.
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  • Study Testing if e-Nose Can Detect Pulmonary Arterial Hypertension by Smelling
  • (Lung Disease News) Researchers have developed an electronic nose, called e-nose, that may be capable of diagnosing pulmonary arterial hypertension (PAH) by “sniffing” it on a person’s breath. A large study evaluating the e-nose’s ability to detect PAH is now recruiting both patients and healthy individuals to serve as controls.
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  • September 12, 2016
  • Adempas Aids PAH Patients Not Improving on PDE5i Therapy, Clinical Study Finds
  • (Pulmonary Hypertension News) Adempas, which was approved by the U.S. Food and Drug Administration for PAH treatment in 2013,  stimulates a molecule known as soluble guanylate cyclase. Several clinical trials have shown that the drug is effective in managing PAH. Now, researchers assessed if Adempas might also aid patients wishing to switch from PDE5i treatment.
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  • Severe Pulmonary Arteriopathy Is Associated with Persistent Hypoxemia after Pulmonary Endarterectomy in CTEPH
  • (PLOS One) Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by occlusion of pulmonary arteries by organized chronic thrombi. Persistent hypoxemia and residual pulmonary hypertension (PH) following successful pulmonary endarterectomy (PEA) are clinically important problems; however, the underlying mechanisms remain unclear. We have previously reported that residual PH is closely related to severe pulmonary vascular remodeling and hypothesize that this arteriopathy might also be involved in impaired gas exchange. The purpose of this study was to evaluate the association between hypoxemia and pulmonary arteriopathy after PEA.
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  • September 9, 2016
  • Pulmonary Hypertension Due to Common Respiratory Conditions: Classification, Evaluation and Management Strategies
  • (Journal of Clinical Medicine) Pulmonary hypertension (PH) due to chronic respiratory disease and/or hypoxia is classified as World Health Organization (WHO) Group III pulmonary hypertension. The patients most commonly encountered in clinical practice with group III PH include those with chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disordered breathing. The purpose of this review is to outline the variable clinical significance of pulmonary hypertension in the most common pulmonary disease states and how a clinician may approach the management of these patients.
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  • September 8, 2016
  • Can Pulmonary Hypertension Be Detected in Breath? E-Nose Thinks So
  • (Pulmonary Hypertension News) “The gold standard for the diagnosis of PAH is right heart catheterism, which can make the right diagnosis, but it is invasive, risky and unsuitable for widespread screening,” said Cohen-Kaminsky in a press release. “The e-nose is a non-invasive and safe detection method that means general screening of PAH could eventually be made available.”
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  • Be aware of fraudulent stem cell treatments
  • (Foster.com) Recently, a patient of a local physician spent $15,000 on a bogus stem cell treatment for COPD in Florida. The patient pursued this option despite warnings from his physician and several other experts. The therapy did not work, and now the patient and his family are out a substantial sum that they could not afford to lose. People with chronic illness and the elderly are desperate for hope for a cure for diseases that physicians know have no cure. For that reason, it is important to get the word out to the public so that unscrupulous pseudo-medical personnel cannot prey on innocent desperate people.
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  • September 7, 2016
  • Prognostic Factors for Pulmonary Hypertension Include Gender, Age, Chronic Diseases
  • (Pulmonary Hypertension News) Researchers used data from the Longitudinal Health Insurance Database, a subset of the National Health Insurance (NHI) database. They created two groups from the data: the PH group, with information relative to 1,092 newly identified patients between 1999 and 2011, and the control group, with data on 8,736 healthy people.
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  • September 6, 2016
  • Arginase Inhibition Reverses Endothelial Dysfunction, Pulmonary Hypertension, and Vascular Stiffness in Transgenic Sickle Cell Mice
  • (Anesthesia and Analgesia) Arginase inhibition improves NO bioavailability and thereby attenuates systemic and pulmonary vascular endothelial dysfunction in transgenic mice with SCD. Therefore, arginase is a potential therapeutic target in the treatment of cardiovascular dysfunction in SCD.
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  • September 5, 2016
  • In Certain CTEPH Patients, Vascular Remodeling May Lead To Hypoxemia
  • (Pulmonary Hypertension News) PEA, a surgical procedure that removes the organized pulmonary blood clots, has been shown to improve the condition of CTEPH patients, showing benefits in blood flow, cardiac function, and the six-minute walk distance test.
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  • September 2, 2016
  • Biomarkers of tissue remodeling predict survival in patients with pulmonary hypertension
  • (International Journal of Cardiology) Matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) are involved in vascular and right ventricular remodeling in pulmonary hypertension (PH). MMP2, MMP9, TIMP1, and TIMP4 were measured in plasma and their potential as biomarkers for PH was evaluated.
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  • September 1, 2016
  • Endothelin receptor antagonists for persistent pulmonary hypertension in term and late preterm infants
  • (Cochrane Library) Some newborn babies develop abnormally high blood pressure in the arteries of the lung. This condition is called persistent pulmonary hypertension of the newborn (PPHN). Babies with PPHN present with breathing difficulties and low oxygen levels.Traditionally such babies are managed with respirators and administration of a special gas called nitric oxide. In many cases, babies do not improve in spite of these measures. A new class of drugs called endothelin receptor antagonists are being tested for PPHN.
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  • Karos Reports Positive Safety Data from Phase 1 Trials of PAH Therapy
  • (Pulmonary Hypertension News) Karos Pharmaceuticals, a start-up focused on the selective modulation of peripheral serotonin synthesis, recently reported positive results from its Phase 1 clinical trials evaluating KAR5585, a compound being developed to treat orphan diseases such as pulmonary arterial hypertension (PAH) and carcinoid syndrome.
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  • August 31, 2016
  • Vascular stiffness mechanoactivates YAP/TAZ-dependent glutaminolysis to drive pulmonary hypertension
  • (JCI) Dysregulation of vascular stiffness and cellular metabolism occurs early in pulmonary hypertension (PH). However, the mechanisms by which biophysical properties of the vascular extracellular matrix (ECM) relate to metabolic processes important in PH remain undefined. In this work, we examined cultured pulmonary vascular cells and various types of PH-diseased lung tissue and determined that ECM stiffening resulted in mechanoactivation of the transcriptional coactivators YAP and TAZ (WWTR1). YAP/TAZ activation modulated metabolic enzymes, including glutaminase (GLS1), to coordinate glutaminolysis and glycolysis. Glutaminolysis, an anaplerotic pathway, replenished aspartate for anabolic biosynthesis, which was critical for sustaining proliferation and migration within stiff ECM. In vitro, GLS1 inhibition blocked aspartate production and reprogrammed cellular proliferation pathways, while application of aspartate restored proliferation. In the monocrotaline rat model of PH, pharmacologic modulation of pulmonary vascular stiffness and YAP-dependent mechanotransduction altered glutaminolysis, pulmonary vascular proliferation, and manifestations of PH. Additionally, pharmacologic targeting of GLS1 in this model ameliorated disease progression. Notably, evaluation of simian immunodeficiency virus–infected nonhuman primates and HIV-infected subjects revealed a correlation between YAP/TAZ–GLS activation and PH. These results indicate that ECM stiffening sustains vascular cell growth and migration through YAP/TAZ-dependent glutaminolysis and anaplerosis, and thereby link mechanical stimuli to dysregulated vascular metabolism. Furthermore, this study identifies potential metabolic drug targets for therapeutic development in PH.
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  • 2 Therapies Intended for Other Diseases May Work to Treat PH
  • (Lung Disease News) The team, led by Dr. Stephen Y. Chan, director of the UPMC Center for Pulmonary Vascular Biology and Medicine at the Vascular Medicine Institute at Pittsburg, discovered that the stiffening and hardening of lung arteries that causes PH triggers the activation of two signaling molecules, called YAP and TAZ. These molecules, in turn, activate a protein called GLS1, which plays a role in energy production and energy usage in the cells of lung vessels.
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  • August 30, 2016
  • New Understanding of Pulmonary Hypertension Leads to Promising Drug Targets
  • (UPMC) A groundbreaking new study led by researchers from the University of Pittsburgh and UPMC has identified a new group of compounds that could have robust effects in treating pulmonary hypertension (PH), an enigmatic but sometimes fatal disease of the blood vessels of the lungs that currently has no cure. The findings, which were published in the Journal of Clinical Investigation, highlight the use of these drugs to alter vessel stiffness and its downstream control of metabolism, a link previously unknown for people suffering from the progressive disease.
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  • High-Fat Diet in Diabetic Moms May Add to Risk of Pulmonary Hypertension in Newborn
  • (Pulmonary Hypertension News) To better understand the possible association between obesity, diabetes, and PPHN, a research team at the University of South Dakota studied pregnant rats with gestational diabetes being fed a high-fat diet. They found that these rats gave birth to offspring with pulmonary complications, which lasted the three weeks they were alive.
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  • Experts seek origin of pulmonary hypertension to design treatment
  • (Trib Live) Dr. Stephen Chan, director of UPMC's Center for Pulmonary Vascular Biology and Medicine, is leading a team using new strategies to target the origins of the disease in search for a cure.  In a paper published in the Journal of Clinical Investigation, he outlines how two compounds, known as verteporfin and CH-839, show promise in impeding the chain of events that leads from initial stiffness in the arteries to changes in metabolism to heart failure. Doctors have used the compounds to treat cancer and macular degeneration, an eye disease.
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  • August 29, 2016
  • Blocking a Stress-response Protein Found to Treat PH in Animal Model
  • (Pulmonary Hypertension News) People with pulmonary arterial  hypertension (PAH) have higher than usual levels of a protein produced in cells in response to shock or stress — called heat shock protein 90 or HSP90 — and diminishing those levels helped to prevent pulmonary hypertension from progressing in a rat model of the disease, researchers reported.
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  • 2 Possible New Treatments for Pulmonary Hypertension ID’d in Study
  • (Pulmonary Hypertension News) Researchers identified two new drugs, both signaling inhibitors, that could aid in the treatment of pulmonary hypertension (PH) by altering vessel stiffness and its downstream control of metabolism. Their work also suggests that a previously unknown link exists between vessel hardening and energy production in the disease.
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