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.
- February 11, 2016
- Medical Mondays: CTEPH
In this week’s Medical Monday video, the focus is on CTEPH, or chronic thromboembolic pulmonary hypertension. This is a condition that arises from repeated or unresolved pulmonary embolism – or clots – in the pulmonary arteries in the lungs. Dr. Patricia George, a pulmonologist at UPMC, explains the symptoms of CTEPH, who’s at risk, why patients should seek treatment at a certified CTEPH center, and how UPMC specializes in the treatment of this condition.
- Anti-U1 RNP positivity linked to lower mortality rate in patients with CTD- and SSc-associated PAH
Patients with connective tissue disease and systemic sclerosis who were positive for anti-U1 RNP antibodies had a decreased mortality risk, according to recently published research.
- February 10, 2016
- The Right Ventricle: A Not-So-Innocent Bystander in Pulmonary Hypertension Due to Left Heart Disease
(PHA Online University)
The International Right Heart Foundation Working Group recently proposed a comprehensive definition of right heart failure as: “a clinical syndrome due to an alteration of structure and/or function of the right heart circulatory system that leads to suboptimal delivery of blood flow (high or low) to the pulmonary circulation and/or elevated venous pressures—at rest or with exercise.” Although the RV is a key component of the right heart system (and our focus), it is important to remember that unfavorable alterations of any component of the circulation from the systemic veins up to the pulmonary capillaries can result in right heart failure symptoms.
- February 9, 2016
- High Frequency of Pulmonary Hypertension-Causing Gene Mutation in Chinese Patients with Chronic Thromboembolic Pulmonary Hypertension
The pathogenesis of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown. Histopathologic studies revealed that pulmonary vasculature lesions similar to idiopathic pulmonary arterial hypertension (PAH) existed in CTEPH patients as well. It’s well-known that genetic predisposition plays an important role in the mechanism of PAH. So we hypothesized that PAH-causing gene mutation might exist in some CTEPH patients and act as a background to facilitate the development of CTEPH.
- February 8, 2016
- Ambrisentan avoids sildenafil drug interaction in PAH patients
Researcher Keiichi Odagiri (Hamamatsu University School of Medicine, Japan) and team found that patients’ plasma concentration of sildenafil was significantly lower if given with bosentan, compared with ambrisentan.
- Overview of WHO Group 2 Pulmonary Hypertension Due to Left Heart Disease
(PHA Online University)
Identifying LHD as the cause of PH is critically important because it determines the correct approach to management, which is optimal treatment of the underlying LHD with evidence-based and/or standard-of-care pharmacologic or surgical therapies. In patients presenting with PH-LHD, there is currently no role for treatment with pulmonary arterial hypertension (PAH)–specific therapies and, with few exceptions, they should not be administered because they are costly, lack efficacy, and in some cases, are known to increase morbidity and mortality.
- February 7, 2016
- PAH Patients with BMPR2 Mutations Likely to Have Severe Disease Progression
(Pulmonary Hypertension News)
Patients with pulmonary arterial hypertension (PAH) carrying mutations in the bone morphogenetic protein receptor type II (BMPR2) gene have a worse prognosis, namely decreased survival rates and higher risk for lung transplantation. The findings were in a study titled “BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis” and published in THE LANCET Respiratory Medicine journal.
- February 6, 2016
- Actelion receives positive CHMP opinion for Uptravi (selexipag) for the long-term treatment of PAH
The CHMP recommended that the European Commission approves Uptravi for the long-term treatment of pulmonary arterial hypertension (PAH) in adult patients with WHO functional class (FC) II-III, either as combination therapy in patients insufficiently controlled with an endothelin receptor antagonist (ERA) and/or a phosphodiesterase type 5 (PDE-5) inhibitor, or as monotherapy in patients who are not candidates for these therapies. Efficacy has been shown in a PAH population including idiopathic and heritable PAH, PAH associated with connective tissue disorders, and PAH associated with corrected simple congenital heart disease.
- February 5, 2016
- PAH Oral Prostacyclin Analog Drugs, Like Treprostinil, Are Improving But Have Way to Go
(Pulmonary Hypertension News)
Prostacyclin analogs have substantially improved pulmonary arterial hypertension (PAH) treatment, but the use of these drugs is hampered by the difficulties and risks of treating patients with continuous intravenous infusions. A new overview highlighted the development of oral prostacyclins, focusing on the drug treprostinil (Orenitram).
- February 3, 2016
- Transfemoral TAVR shows reductions in mortality, paravalvular leak
Researchers evaluated 1,063 participants in the PARTNER trial nonrandomized continued access registry who underwent transfemoral TAVR for severe native trileaflet aortic stenosis. Participants were grouped into tertiles based on procedure date: tertile 1 (n = 353; March 24, 2009-July 21, 2010); tertile 2 (n = 355; July 22, 2010-March 10, 2011); and tertile 3 (n = 355; March 11, 2011-Jan. 10, 2012).
- February 2, 2016
- When patients and drug makers align on a cause, whose best interests are at play?
(The Globe Unlimited)
“There is an important role for patient groups, no question,” said Steve Morgan, an expert in drug policy at the University of British Columbia. “There is also, unfortunately, a conflict of interest that a lot of patient groups find themselves in as they accept more and more money from pharmaceutical manufacturers.”
- February 1, 2016
- Fiber-Rich Diet May Boost Lung Function
Fruit and vegetable consumption was more strongly associated with higher lung function than consumption of whole grains in the analysis, and this association was independent of antioxidant intake, which has previously been shown to be positively associated with lung function
- January 31, 2016
- A case of a patient with pulmonary hypertension associated with multiple endocrine dysfunctions and connective tissue disorder
Pulmonary hypertension in association with connective tissue disorders, thyroid dysfunctions and hyperestroginism has been reported. However, the presence of pulmonary hypertension in multiple endocrine dysfunctions (i.e. pituitary adenoma, endometriosis and ovarian cysts, thyroid dysfunction) with connective tissue disorder has rarely been described. This is a case of the coexistence of pulmonary hypertension in a patient with functional pituitary adenoma, thyroid cysts and goiter with endometriosis and connective tissue disease with a diagnostic difficulty faced in presence of long standing bronchial asthma. The case illustrates the association of pulmonary hypertension in the setting of endocrine dysfunctions and connective tissue disease, and the effect on its course.
- January 30, 2016
- Peripheral pulmonary artery stenosis as a cause of pulmonary hypertension in adults
(University of Chicago Press)
Peripheral pulmonary artery stenois (PPAS) is an underrecognized condition in the adult population. PPAS can lead to pulmonary hypertension but is likely misdiagnosed as either idiopathic pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.
- January 29, 2016
- New Metabolites Associated With Hemodynamic Indicators of Right Ventricular-Pulmonary Vascular Dysfunction Discovered
New metabolites associated with hemodynamic indicators of right ventricular-pulmonary vascular (RV-PV) dysfunction have been discovered, according to a study recently published by the Journal of American College of Cardiology.
- Incident pulmonary arterial hypertension associated with greater risk for disease progression
Simonneau and colleagues evaluated 257 patients enrolled in the Study with an Endothelin Receptor Antagonist in Pulmonary Arterial Hypertension to Improve Clinical Outcome (SERAPHIN) study and divided them into incident (≤ 6 months; n = 110 patients) and prevalent (> 6 months; n = 157 patients) groups based on their time to diagnosis, according to the abstract. The researchers then assigned patients either 3 mg or 10 mg of macitentan, or placebo. They used a Cox regression analysis to determine mortality or hospitalization due to pulmonary arterial hypertension as well as risk for morbidity or mortality in these groups.
- January 28, 2016
- BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis
In the year 2000, mutations in the BMPR2 gene were identified as the major genetic cause of pulmonary arterial hypertension (PAH). Some small studies have examined the effect of BMPR2 mutations on the presentation, haemodynamic profile, and outcomes in patients with PAH. These studies suggested that those with BMPR2 mutations present at a younger age with more severe derangements of cardiopulmonary haemodynamics. Due to a lack of statistical power, lack of adjustment for important factors such as age and sex, and confounding from inclusion of prevalent cases without necessary adjustments in many of these studies, the effect of BMPR2 mutations on long-term outcomes has not been reliably established.
- January 27, 2016
- Pulmonary Hypertension Often Missed in its Early Stages
With advances in diagnosis and treatment options, pulmonary hypertension is more treatable today than ever before. The biggest challenge, says Ronald Zolty, MD, cardiologist at Nebraska Medicine, is getting an early diagnosis.
- January 26, 2016
- Actelion receives Health Canada approval for Uptravi
- £1.5m to back Cambridge firm developing revolutionary treatment for respiratory condition
Morphogen-IX has gained the seed funding to develop a new treatment for pulmonary arterial hypertension (PAH). This potential therapy is based on 15 years of work by Professor Nick Morrell and his team at Cambridge University's department of medicine at Addenbrooke's Hospital and could provide the first disease-modifying approach to the condition.
- January 24, 2016
- CardioBrief: Why Guidelines Are Bad for Science
By their very nature, guidelines present the illusion of successful science, the appearance that clarity and understanding has been achieved by the experts. When this happens, science is the victim.
- January 23, 2016
- Editors Deliver Major Victory to Open Data Movement
Harlan Krumholz, MD, principal investigator of the Yale University Open Data Access (YODA) Project expressed considerable enthusiasm for the development:
"This is a remarkable day; the medical editors showed leadership in demonstrating that we medical scientists must put the interests of society first. There are a lot of details to work out -- but they have given substantial impetus to the open science movement and to the efforts to have medical research be transparent. If this proposal is accepted, then the medical science world will be forever transformed for the better."
- January 22, 2016
- Potentially transformative therapy for rare heart disease to be progressed by Morphogen-IX
Morphogen-IX, a new Cambridge-based biotech company, has gained seed funding to develop a new treatment for pulmonary arterial hypertension (PAH) that directly targets the major pathway implicated in human genetic studies.
- January 21, 2016
- Lupus affects lungs, leads to pleuritis, pneumonitis and pulmonary hypertension
(Bel Marra Health)
When a person has a lung infection it usually affects their airway and lung tissue, but with lupus it seems that it can impact all compartments of the lungs and include pleuritis, which is inflammation of the linings around the lung, as well as pulmonary hypertension (high blood pressure that occurs in the arteries in the lungs). Those with lupus and lung problems can also get pneumonitis, inflammation of the walls of the alveoli in the lungs, normally caused by a virus.
- January 20, 2016
- Lung cell found to act as sensor, regulator of immune response
An uncommon and little-studied type of cell in the lungs has been found to act like a sensor, linking the pulmonary and central nervous systems to regulate immune response in reaction to environmental cues.
- January 19, 2016
- Formulation scientists to rescue patients with pulmonary arterial hypertension
Researchers from the Pharmacy and the Chemical and Biomolecular Engineering departments of the National University of Singapore (NUS) and the Agency for Science, Technology and Research (A*STAR) have designed formulations of controlled release microspheres which release the medicine over a prolonged period of time and thereby reduce the number of doses a patient needs to take. It provides a friendly and convenient alternative treatment for pulmonary arterial hypertension (PAH) which is a rare but serious illness and worsens if untreated. There is no known cure for PAH but with appropriate care and treatment, its progression will be slowed with alleviated symptoms.
- January 18, 2016
- Video Feature: Idiopathic Pulmonary Fibrosis
Pulmonary fibrosis has been a major challenge to treat. Why? Who gets idiopathic fibrosis? What is new in treatment? What should physicians and patients consider when choosing between pirfenidone (Esbriet) and nintedanib (Ofev) for therapy?
- January 17, 2016
- Therapeutic Potential of microRNA Modulation in PulmonaryArterialHypertension
MicroRNAs have emerged as key players of gene regulation during development and disease states like cancer and cardiovascular diseases. Pulmonary arterial hypertension (PAH), a vascular disease characterized by pulmonary resis-tance vessel occlusion is not spared by microRNA implication. This is not surprising since PAH shares common aber-rantly activated pathways with cancers that lead to proliferation and survival of pulmonary arterial smooth muscle cells, among others, within the artery wall and narrowing the lumen. Recent studies demonstrated the role of miR-204 and miR-206 in pulmonary artery smooth muscle cell (PASMC) proliferation. Other microRNAs, such as miR-145, miR-21 and the miR17/92 cluster, have been associated with the disrupted BMPR2 pathway. During the last couple of years, the number of studies on the role of microRNA in PAH has broadened, defining it clearly as a HOT TOPIC. This current review pre-sents an overview of the most recent knowledge as well as future possibilities. The use of microRNA therapies is still un-certain and poorly applied in the clinical setting yet. It is still critical to increase the knowledge and the translational po-tential of this HOT TOPIC to make it become a HOPE TOPIC.
- January 16, 2016
- Pulmonary Hypertension
An in-depth PowerPoint presentation by Prof AKM Mosharraf Hossain PhD FCPS Professor of Respiratory Medicine BSMMU that provides a very good overview of Pulmonary Hypertension, from it's causes thru it's various treatments.
- January 15, 2016
- Genetic counselling in a national referral centre for pulmonary hypertension.
Genetic causes of pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease (PVOD) have been identified, leading to a growing need for genetic counselling.Between 2003 and 2014, genetic counselling was offered to 529 PAH and 100 PVOD patients at the French Referral Centre for Pulmonary Hypertension. Mutations in PAH-predisposing genes were identified in 72 patients presenting as sporadic PAH (17% of cases; 62 mutations in BMPR2, nine in ACVRL1 (ALK1) and one in ENG) and in 94 patients with a PAH family history (89% of cases; 89 mutations in BMPR2, three in ACVRL1 (ALK1) and two in KCNK3). Bi-allelic mutations in EIF2AK4 were identified in all patients with a family history of PVOD (n=19) and in seven patients (8.6%) presenting as sporadic PVOD. Pre-symptomatic genetic diagnosis was offered to 272 relatives of heritable PAH patients, identifying mutations in 36.4% of them. A screening programme is now offered to asymptomatic mutation carriers to detect PAH in an early phase and to identify predictors of outcomes in asymptomatic BMPR2 mutation carriers. BMPR2 screening allowed us to offer pre-implantation diagnosis to two couples with a BMPR2 mutation.Genetic counselling can be implemented in pulmonary hypertension centres.
- January 14, 2016
- BETA-3 Adrenoceptor Agonists for the Treatment of Pulmonary Hypertension
(Free Patents Online)
There has been little research on β3 adrenergic receptors in the field of cardiovascular diseases. Stimulation of these receptors is associated with the production of nitric oxide and the relaxation of vascular tone. In a study with rat lung samples, stimulation of β3 adrenergic receptors produced dose-dependent relaxation (Dumas 1998). In a functional study of rings of pulmonary artery extracted from dogs, an increase of relaxation with selective stimulation of β1, β2 and β3 adrenergic receptors was observed (Tagaya 1999). However, in another study in which pulmonary arteries were extracted from control mice and from mice with hypoxia-induced PH, increase of relaxation with selective stimulation of β3 receptors was not observed (Leblais, 2008).
- January 13, 2016
- Left ventricular diastolic dysfunction in PH predicts functional capacity and clinical worsening: a tissue phase mapping study
(Journal of Cardiovascular Magnetic Resonance)
The function of the right and left ventricles is intimately related through a shared septum and pericardium. Therefore, right ventricular (RV) disease in pulmonary hypertension (PH) can result in abnormal left ventricular (LV) myocardial mechanics. To assess this, we implemented novel cardiovascular magnetic resonance (CMR) tissue phase mapping (TPM) to assess radial, longitudinal and tangential LV myocardial velocities in patients with PH.