Author Archives: Margarida Azevedo, MSc

Same Immune Cell Dysfunction, But Not Its Cause, Found in All Types of PAH

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,…

PAH Drug, Opsumit, Seen to Prevent and Treat Lung Disease in a Mouse Model of Scleroderma

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…

Portopulmonary Hypertension Seen as Important Prognosis Parameter in Liver Transplant Candidates

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 PAH, Tissue Phase Mapping of Left Ventricle May Predict Clinical Outcomes

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…

New PAH Drug Delivery System Receives EC Certification

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…

Proposed PAH Drug Delivery System Allows for Controlled Release of Medicine

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…

New PAH Drug from Actelion Approved by FDA

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…

CTPER-index May Be a Potential CTEPH Diagnostic Tool

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…

Intrapulmonary Vascular Dilatations Often Found in POPH Patients

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…