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.
- October 13, 2015
- Prostacyclin and Oral Vasodilator Therapy in Sarcoidosis-Associated Pulmonary Hypertension
Many patients with severe sarcoidosis-associated pulmonary hypertension (SAPH) showed significant hemodynamic and clinical improvement on long-term IV or subcutaneous prostacyclin therapy and had survival outcomes similar to patients with moderate SAPH on oral vasodilator therapy.
- Endothelial NO-Synthase Gene-Enhanced Progenitor Cell Therapy for Pulmonary Arterial Hypertension: The PHACeT Trial.
The Pulmonary Hypertension and Angiogenic Cell Therapy (PHACeT) trial was a phase 1, dose-escalating clinical study of the tolerability of culture-derived endothelial progenitor cells, transiently transfected with endothelial nitric oxide synthase, in patients with PAH refractory to PAH-specific therapies.
- October 12, 2015
- Management of Acute and Chronic Pulmonary Embolism – Part 2
(UPMC Physician Resources)
Video: Drs. Arthur J. Boujoukas, M. Patricia George, and Thomas G. Gleason join together to present on the management of acute and chronic pulmonary embolism.
- Role of the Soluble Epoxide Hydrolase in Hypoxic Pulmonary Vasoconstriction and Pulmonary Vascular Remodeling
The soluble epoxide hydrolase (sEH), which is expressed in pulmonary artery smooth muscle cells, metabolizes cytochrome P450 (CYP) epoxygenase-derived epoxyeicosatrienoic acids (EETs) to their less active diols. Preliminary findings indicate a role of the sEH on hypoxic pulmonary vasoconstriction (HPV) and a vasoconstrictor role of EETs in the pulmonary vasculature.
- October 11, 2015
- Pulmonary Hypertension in Children – a Practical Approach
Pulmonary hypertension (PH) is mean pulmonary arterial pressure above 25 mmHg at rest. Although considered a rare disease, the prevalence of PH in certain risk groups is higher, from 0.5% in patients with HIV infection to 30% in congenital heart disease (CHD) associating PH. In pulmonary arterial hypertension (PAH) associated with CHD, early changes of the vascular bed are reversible after correction of the defect, but there is a point from where the disease becomes irreversible or progression continues despite correction. Among patients with “operable” and “inoperable” CHD, there is a “gray area” group in between, which is defined by pulmonary vascular resistance equal to 4-8 WU/m and ratio of pulmonary vascular resistance to systemic vascular resistance of 0.3-0.5, measured by cardiac catheterization. In this situation a pulmonary vasoreactivity test is indicated. Pulmonary hypertension is a severe disease with increased morbidity and mortality. Pulmonary hypertension can result in death by decreased cardiac output, and heart failure.
- The Swiss registry for pulmonary arterial hypertension: the paediatric experience
(Archive ouverte UNIGE)
Pulmonary arterial hypertension is a rare disease with a poor prognosis. Epidemiological data are scarce, particularly in the paediatric population. A registry was recently developed in order to collect epidemiological data on patients with pulmonary arterial hypertension (PAH) in Switzerland. This is the first description of the paediatric data.
- October 10, 2015
- Portable bionic lung 'breathes' oxygen directly into the bloodsteam
A portable bionic lung that 'breathes' oxygen directly into the bloodstream is being offered to critically ill patients – handing them valuable extra time while they wait for a transplant.
- Leukotriene B4 Activates Pulmonary Artery Adventitial Fibroblasts in Pulmonary Hypertension
A recent study demonstrated a significant role for leukotriene B4 (LTB4) causing pulmonary vascular remodeling in pulmonary arterial hypertension. LTB4 was found to directly injure luminal endothelial cells and promote growth of the smooth muscle cell layer of pulmonary arterioles.
- October 9, 2015
- The Application of Intravascular Ultrasound to Evaluate Pulmonary Vascular Properties and Mortality in Patients with Pulmonary Arterial Hypertension.
Intra vascular ultrasound (IVUS) is useful in PAH assessment by evaluating pulmonary vascular properties and predicting mortality. The classification of the proximal and distal remodeling type of PAH may be proposed to predict mortality and evaluate the prognosis of patients with PAH in clinical practice.
Exciting PAH research from China. Click headline for more.
- October 8, 2015
Steers grown at altitudes of greater than 1300m, and late fed to fat can suffer from heart failure in the age of only 18 months. had an everage mPAP of 50mmHg.
- October 7, 2015
- Does Circulating IL-17 Identify a Subset of Patients With Idiopathic Pulmonary Arterial Hypertension?
Elevated IL-17A levels are, indeed, detectable in a minor subset of patients with IPAH, but likewise in some healthy subjects. We did not observe associations of IL-17A with disease severity or outcome. Our data support the findings by Hautefort et al1 and warrant the need for larger studies to disclose a more moderate implication of detectable IL-17A level in patients with IPAH.
- October 6, 2015
- Food and Drug Administration Approves New Treatment Combination of Gilead’s Letairis® with Tadalafil for Pulmonary Arterial Hypertension (WHO Group 1)
click headline for press release.
- October 5, 2015
- Therapeutic potential of soluble guanylate cyclase modulators in neonatal chronic lung disease.
(American Journal of Physiology)
Recent studies highlight the therapeutic potential of sGC modulators in the treatment of preterm infants for respiratory distress with supplemental oxygen. Such treatment is aimed at improving aberrant alveolar and vascular development of the neonatal lung, and preventing the development of wheezing and asthma in survivors of premature birth. In addition, these studies highlight the suitability of fetal human airway SMC's as a translational model for pathological airway changes in the neonate.
- October 4, 2015
- ACP Guideline Calls for Smarter Testing in PE Evaluation
Usage of CT imaging and D-dimer testing in diagnosing pulmonary embolism (PE) should be decided on the basis of "pretest" likelihood that patients genuinely have the condition, the American College of Physicians (ACP) said in a new practice guideline.
- October 3, 2015
- Why there is a need to discuss pulmonary hypertension other than pulmonary arterial hypertension?
(Baishideng Publishing Group Inc.)
Pulmonary arterial hypertension (PAH) is a rare disease that concerns a small population of patients. Recently, there has been a significant number of research, publications and novel therapies concerning PAH. However, pulmonary hypertension (PH), that concerns a much larger population of patients with common diseases such as lung and left heart diseases (LHD), is generally overlooked despite the fact that it significantly affects the prognosis of these patients. This editorial underlines the need for further research in regard to the pathogenesis and novel therapies for PH related to lung and LHD.
To read the full text ahead of print manuscript in Word format, please follow this link: http://www.wjgnet.com/esps/DownloadFile.aspx?Type=M&MsId=19900&FilePath=UserFiles\Manuscript\00023448\19900-Review.doc
- October 2, 2015
- B-Lines in Assessment of Pulmonary Hypertension in Patients With Interstitial Lung Diseases: Feasibility of Transthoracic Lung Sonographic Signs.
The number of B-lines is useful in assessment of pulmonary hypertension, especially when tricuspid regurgitation and pulmonary valve regurgitation do not exist or cannot be satisfactorily measured by Doppler echocardiography.
- Calcilytics Explored as Potential Drug Candidates for Pulmonary Arterial Hypertension Treatment
(Lung Disease News)
In a paper recently published in the PLOS ONE journal entitled “Inhibition of Excessive Cell Proliferation by Calcilytics in Idiopathic Pulmonary Arterial Hypertension,” researchers from Kinjo Gakuin University in Japan demonstrated that excessive proliferation of pulmonary arterial smooth muscle cells (PASMC) in patients with pulmonary arterial hypertension (PAH) could be regulated by means of drug-based calcilytics
- October 1, 2015
- An initial combined treatment for pulmonary arterial hypertension provides better long-term results
A study published in the New England Journal of Medicine (NEJM) shows that the combination of two drugs (ambrisentan and tadalafil) is more effective than the use of one of these agents to treat pulmonary arterial hypertension. The combination therapy improves very significantly the outcome of patients with this disease and reduces by 50% the risk of clinical failure. Dr. Joan Albert Barberà, pulmonologist at Hospital Clínic and head of the IDIBAPS team Physiopathological mechanisms of respiratory illnesses is one of the authors of this study and coordinator of the trial in Spain.
- September 30, 2015
- Study Demonstrates Benefit of Cytori’s Scleroderma Treatment After One Year
(Pulmonary Hypertension News)
Cytori Therapeutics recently published a report of 12-month data from the Scleradec-I clinical trial studying hand dysfunction in people with scleroderma. Findings from the 12 patient trial, which was led by Dr. Brigitte Granel and Dr. Guy Magalon of the Assistance Publique des Hôpitaux de Marseille in France, is titled “Autologous adipose-derived stromal vascular fraction in patients with systemic sclerosis: 12-month follow-up,” and was published August 4th in the journal Rheumatology.
- September 29, 2015
- Low-Intensity Exercise Appears Safe for Pulmonary Hypertension
(Renal & Urology News)
Exercise has a positive effect on several measures of heart function as well as overall quality of life for patients with pulmonary hypertension, according to research published recently in Circulation: Heart Failure.
- September 28, 2015
- Study of INOpulse for Pulmonary Arterial Hypertension Reveals Positive Data
(Pulmonary Hypertension News)
Bellerophon Therapeutics recently announced that INOpulse, a type of nitric oxide that helps blood vessels expand, is effective in treating Pulmonary Arterial Hypertension (PAH) based on middle-stage data from a Phase 2 trial. The US Food and Drug Administration (FDA) has issued a Special Protocol Assessment (SPA), which will help to speed up the Phase 3 INOpulse studies.
- September 27, 2015
- Guidelines for Diagnosis and Treatment of Pulmonary Hypertension
(American College of Cardiology)
The article discusses the key points to remember from the European Society of Cardiology/European Respiratory Society guidelines for the diagnosis and treatment of pulmonary hypertension (PH).
- September 26, 2015
- Inhibition of Excessive Cell Proliferation by Calcilytics in Idiopathic Pulmonary Arterial Hypertension
Idiopathic pulmonary arterial hypertension (IPAH) is a rare and progressive disease of unknown pathogenesis. Vascular remodeling due to excessive proliferation of pulmonary arterial smooth muscle cells (PASMCs) is a critical pathogenic event that leads to early morbidity and mortality. The excessive cell proliferation is closely linked to the augmented Ca2+ signaling in PASMCs. More recently, we have shown by an siRNA knockdown method that the Ca2+-sensing receptor (CaSR) is upregulated in PASMCs from IPAH patients, involved in the enhanced Ca2+ response and subsequent excessive cell proliferation. In this study, we examined whether pharmacological blockade of CaSR attenuated the excessive proliferation of PASMCs from IPAH patients by MTT assay. The proliferation rate of PASMCs from IPAH patients was much higher (~1.5-fold) than that of PASMCs from normal subjects and patients with chronic thromboembolic pulmonary hypertension (CTEPH). Treatment with NPS2143, an antagonist of CaSR or calcilytic, clearly suppressed the cell proliferation in a concentration-dependent manner (IC50 = 2.64 μM) in IPAH-PASMCs, but not in normal and CTEPH PASMCs. Another calcilytic, Calhex 231, which is structurally unrelated to NPS2143, also concentration-dependently inhibited the excessive proliferation of IPAH-PASMCs (IC50 = 1.89 μM). In contrast, R568, an activator of CaSR or calcimimetic, significantly facilitated the proliferation of IPAH-PASMCs (EC50 = 0.33 μM). Similar results were obtained by BrdU incorporation assay. These results reveal that the excessive PASMC proliferation was modulated by pharmacological tools of CaSR, showing us that calcilytics are useful for a novel therapeutic approach for pulmonary arterial hypertension.
- September 25, 2015
- The Reversal of Pulmonary Vascular Remodeling Through Inhibition of p38MAPK-alph
(American College of Cardiology)
It is increasingly recognized that inflammation plays an important role in the development and progression of pulmonary hypertension (PH)2. A number of diseases with inflammatory phenotypes have been associated with the development of PH and pulmonary vascular remodeling and indeed anti-inflammatory therapies can in some cases prevent or even reverse disease...
This study underpins current feelings that inflammation is an important biological process in the development and progression of pulmonary hypertension. It suggests a direct link between increased levels of IL-6 and pulmonary hypertension mediated through the pro-inflammatory p38MAPK pathway. It also confirms that the pulmonary artery fibroblast is an important contributing cell to the remodeling process. Inhibition of this p38 pathway may, in turn, allow direct targeting of one or more inflammatory pathways driving the biology of pulmonary hypertension and provide a novel treatment strategy in this disease
- Bayer HealthCare Tests Chemical Libraries for New PAH Treatment
Research scientists at Bayer HealthCare AG in Germany are optimizing a new chemical inhibitor for pulmonary arterial hypertension (PAH) treatment. The team of medicinal chemists generated a library of compounds centered around inhibiting human neutrophil elastase (HNE), a key driver of inflammation in PAH. After generating molecules that successfully inhibited HNE, the team treated rats with monocrotaline-induced PAH and found an attenuation of symptoms.
- September 24, 2015
- Mosaic Pattern of Lung Attenuation on Chest CT Scans Detected in Some Pulmonary Hypertension Patients
(Lung Disease News)
A new study identified a mosaic pattern of lung attenuation on chest CT scans detected in some Pulmonary Hypertension patients, particularly those patients with CTEPH. The study, entitled “Mosaic Pattern of Lung Attenuation on Chest CT in Patients with Pulmonary Hypertension,” was published in the journal Diseases.
- Pulmonary Hypertension Study Links Sickle Cell Anemia to PH
In a recent collaborative research project published in the PLOS one journal entitled “Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia,” researchers suggest that patients suffering from sickle cell anemia (SCA) have higher risks for developing pulmonary hypertension (PH).
- September 23, 2015
- Reata to Present Data on Bardoxolone Methyl Therapy for PAH
Reata Pharmaceuticals has announced that it will present Phase 2 results from a trial of its investigational therapy bardoxolone methyl in treating pulmonary arterial hypertension (PAH).
- September 22, 2015
- Prolonged vasodilatory response to nanoencapsulated sildenafil in pulmonary hypertension
(Nano Med Journal)
Direct vasodilator delivery to the airways enables a selective therapy of pulmonary hypertension (PH). However, short-term effects of the applied medication require multiple daily inhalations. Controlled release formulations (polymeric nanomedicines) offer the potential of prolonging drug effects within the respiratory tract, thereby reducing the number of necessary inhalations.
- September 21, 2015
- High False Negative Rate in Detecting Scleroderma Lung Disease Associated With Pulmonary Function Tests
(Scleroderma News Today)
In a recent study published in the journal Arthritis & Rheumatology, entitled, “Pulmonary Function Tests: High Rate of False Negatives in the Early Detection and Screening of Scleroderma Interstitial Lung Disease,” a team of researchers from the University Hospital Zurich led by Yossra A. and Suliman, M. found that the use of pulmonary function tests (PFTs) is linked with high false negative rates for identification of scleroderma-associated interstitial lung disease (SSc-ILD).
- Cases of pulmonary hypertension grow in children
While the disease is uncommon in children, researchers from the Johns Hopkins School of Medicine have found that more and more kids are being hospitalized for the disease
- September 20, 2015
- Macitentan for the Treatment of PAH: A Review of the Literature for the Recently Approved ERA
The pharmacokinetic profile, clinical efficacy, and safety of this recently approved dual endothelin receptor antagonist for the treatment of pulmonary arterial hypertension (PAH) are reviewed.
- September 19, 2015
- Mosaic Pattern of Lung Attenuation on Chest CT in Patients with Pulmonary Hypertension
A mosaic pattern of lung attenuation on chest computed tomography (CT) may be due to various etiologies. There is limited published data on CT results when used to evaluate pulmonary hypertension (PH). We retrospectively studied the frequency of mosaic pattern in patients with PH and the cause of the PH by diagnostic group, as well as the correlation between the mosaic pattern and the following: demographics, severity of the PH, main pulmonary artery (PA) size, PA/aorta (PA/Ao) ratio, pulmonary function tests (PFT), and ventilation perfusion scan results. Overall, 18% of the cohort had CT mosaic pattern (34/189). Mosaic pattern was present in 17/113 (15%) in Group 1 pulmonary arterial hypertension, 5/13 (28%) in Group 2 pulmonary venous hypertension and 8/50 (16%) in Group 3 PH. Conversely, Group 4 chronic thromboembolic PH was more prevalent in 4/8 (50%). Main PA size, PA/Ao ratio, and segmental perfusion defect were positively associated with mosaic pattern. In contrast, factors such as age, gender, body mass index, functional class, hemodynamic data, and PFT values were not associated with mosaic pattern. Mosaic pattern is not specific as an isolated finding for distinguishing the subtype of PH.
- September 18, 2015
- Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia
One of the most controversial complications of sickle cell disease (SCD) in general and sickle cell anemia (SS) in particular, at the present, is pulmonary hypertension (PH). The controversy centers on determining accurate screening methods and/or identifying predicting factors, if any, of its diagnosis. Moreover, if it is properly diagnosed the controversy extends to determine the best therapeutic interventions. The words “pulmonary hypertension” like “anemia” do not indicate a specific diagnosis but imply a constellation of signs and symptoms that have many possible etiologies. The World Health Organization classifies PH into 5 groups which were collectively referred to as “pulmonary hypertension” with number one being pulmonary arterial hypertension (PAH). Pulmonary hypertension is defined as resting mean pulmonary artery pressure (MPAP) ≥ 25 mmHg determined by right heart catheterization (RHC). Moreover, the hallmark of PAH, besides the elevated MPAP, is a co-existent pulmonary-capillary wedge pressure ≤ 15 mmHg. About 3% of patients with SS develop PAH and the overall prevalence of all types of PH in SS is approximately 6%. Although all types of PH listed in Categories 2–5 may complicate SS, the second most common type of PH in SS is category 2 which is best described as pulmonary venous hypertension (PVH) characterized by elevated level of MPAP to ≥ 25 mmHg and elevated pulmonary capillary wedge pressure to ≥15 mmHg [3–6] most commonly due to left heart disease.
- September 17, 2015
- Pulmonary hypertension in patients with chronic myeloproliferative disorders
Pulmonary hypertension (PH) is a major complication of several haematological disorders. Chronic myeloproliferative diseases (CMPDs) associated with pulmonary hypertension have been included in group five of the clinical classification for pulmonary hypertension, corresponding to pulmonary hypertension for which the aetiology is unclear and/or multifactorial. The aim of this review is to discuss the epidemiology, pathogenic mechanism and treatment approaches of the more common forms of pulmonary hypertension in the context of CMPD's: chronic thromboembolic pulmonary hypertension, precapillary pulmonary hypertension and drug-induced PH.
- Review Highlights Three Therapies on the Market for PAH Management
(Pulmonary Hypertension News)
A recent review, entitled “Medical treatment update on pulmonary arterial hypertension,” presents the latest research for three new drugs that have been approved by the US FDA to treat PAH. The review, which is published in Therapeutic Advances in Chronic Disease, highlights the mechanisms of action for the therapies, as well as the clinical trials that led to their approvals
- September 16, 2015
- Right Heart Parameters In Patients With Left Heart Disease-Associated Pulmonary Hypertension
Dr. Paul Forfia discusses his poster evaluating right heart parameters in patients with left heart disease-associated pulmonary hypertension. His research found the combination of TAPSE (a measure of right heart function) and Doppler notching (an index of high afterload) was robust in predicting diastolic pulmonary pressure gradient (DPG) elevation. Recorded at the 2015 ATS Annual Conference.
- B-Type Natriuretic Peptide: Biomarker of Persistent Pulmonary Hypertension of the Newborn?
BNP had excellent sensitivity and negative predictive value for iNO requirement and above 30 ng/dl maybe a useful prognostic biomarker in PPHN.
- PAH: Targeting Prostycyclin and Endotheliin
Pulmonary arterial hypertension (PAH) affects between 15 and 50 million individuals.1 Remodeling of the pulmonary vasculature, thrombi, and increased vascular resistance and pulmonary arterial pressure (over 25 mm Hg) lead to right heart failure and death if not treated.1-4 PAH may be idiopathic or genetic, or caused by drugs and/or disease.1 The 3-year survival rate for idiopathic or heritable PAH is 74%.
- September 15, 2015
- PAH Clinical Trial Designs Are Evolving, Paving the Way Toward Improved Therapies
(Pulmonary Hypertension News)
The recent announcement of positive results for the AMBITION trial testing combinatorial ambrisentan and tadalafil in patients with pulmonary arterial hypertension (PAH) is bringing more attention to the need to actively recruit participants for clinical trials. Without patients and clinicians interested in trying new treatments for a currently incurable condition, promising new therapies cannot be brought to market for the general PAH population.
- phaware Global Association Launches New Website And High-tech Pulmonary Hypertension Research Focus
(Pulmonary Hypertension News)
Launching today, the Burbank, California-based phaware global association (phaware) is a new non-profit organization and website founded by a group of pulmonary hypertension (PH) awareness advocates. The main purpose of phaware is to reach the general public, news media, and potential donors in order to expand their exposure both nationally and internationally in unprecedented ways.
- September 14, 2015
- Criteria for diagnosis of exercise pulmonary hypertension
(European Respiratory Journal)
The previous definition of exercise pulmonary hypertension (PH) with a mean pulmonary artery pressure (mPAP) >30?mmHg was abandoned because healthy individuals can exceed this threshold at high cardiac output (CO). We hypothesised that incorporating assessment of the pressure–flow relationship using the mPAP/CO ratio, i.e. total pulmonary resistance (TPR), might enhance the accuracy of diagnosing an abnormal exercise haemodynamic response.