In a new study, researchers at the University of Vermont developed a set of synthetic molecules that can successfully modulate the protein kinase 1alpha (PKG 1α), known to be involved in smooth muscle contraction. The protein is a potential therapeutic target for a range of conditions, including pulmonary hypertension.
Researchers found that key immune cells, called regulatory T lymphocytes (Treg), are dysfunctional in all forms of pulmonary arterial hypertension (PAH), but the nature of the alterations differ among subtypes of the disease. Specifically, Treg dysfunction is leptin-dependent in idiopathic PAH (iPAH) and connective tissue disease-associated PAH, and leptin-independent in heritable PAH. Their study,…
Actelion Ltd. was recently recognized for its work in the treatment of rare diseases with the the EURORDIS Company Award 2016, an honor given by the European Organization for Rare Diseases (EURORDIS). The award, given to businesses for pioneering work in drug discovery and development, also acknowledged Actelion’s work with pulmonary…
Researchers at University College London (UCL) and Actelion Pharmaceuticals presented a study investigating the therapeutic effect of macitentan (Opsumit) in a mouse model of pulmonary hypertension associated with systemic scleroderma (SSc). The presentation, titled “Macitentan Responsiveness Supports the Validity of a Murine Model of Pulmonary Hypertension in Scleroderma…
Researchers at the Baskent University Faculty of Medicine in Turkey recently provided an overview of the existing literature regarding portopulmonary hypertension (PoPHT) in patients with advanced liver disease and liver transplant candidates. In these patients, pulmonary hypertension is also defined as PoPHT when pulmonary arterial hypertension (PAH) is associated with…
In a new study, researchers used novel cardiovascular magnetic resonance (CMR) tissue phase mapping (TPM) to determine if right ventricular disease in pulmonary hypertension (PH) patients also resulted in abnormal left ventricular functioning, discovering that left ventricular myocardial velocities are significantly abnormal in PH and also constitute independent predictors of clinical…
Bellerophon Therapeutics recently announced results from the final analysis of the second part of its Phase 2 clinical trial evaluating INOpulse for the treatment of pulmonary arterial hypertension (PAH), supporting the device’s continued evaluation in an upcoming Phase 3 trial. INOpulse is a proprietary device consisting of a pulsative delivery system…
Clinical-stage biotherapeutics company, Bellerophon Therapeutics, Inc., recently announced that its proprietary program device, a new version of the INOpulse drug-device delivery system, has received EC certification with CE marking, meaning that it has met the European Union’s regulatory health, safety, and environmental requirements for a new medical device and can be marketed…
Researchers at the National University of Singapore (NUS) and the Agency for Science, Technology and Research (A*STAR) have developed an alternative treatment system for patients with pulmonary arterial hypertension (PAH), consisting of a controlled-release microparticle formulation that’s delivered through inhalation. The article reporting the system’s development was published in the…
According to a recent announcement, the United States Food and Drug Administration (FDA) approved Actelion Pharmaceuticals’ Uptravi (selexipag) tablets for the treatment of adult patients with pulmonary arterial hypertension (PAH) to delay disease progression and reduce the risk of hospitalization, under orphan drug designation. PAH is a life-threatening disease…
Researchers have developed a computer tomography pulmonary embolism residua index (CTPER-index) that may prove to be a useful tool for diagnosis and treatment decisions regarding chronic thromboembolic pulmonary hypertension and dyspnea. The research paper, “Computed tomography pulmonary embolism residua index (CTPER-index): a simple tool for pulmonary embolism residua description,” was…
In a new study, researchers observed that intrapulmonary vascular dilatations (IPVDs), essential features of hepatopulmonary syndrome (HPS), were more commonly found than expected in a study evaluating patients with portopulmonary hypertension, leading to a recommendation that all such patients be screened for IPVD. The research paper, titled “Intrapulmonary vascular dilatations…
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