News

Activation of a molecule called PPAR-gamma may be a future strategy to prevent pulmonary arterial hypertension (PAH) and other cardiovascular/pulmonary diseases, a study suggests. Findings were reported in the study, “PPARγ agonist pioglitazone reverses pulmonary hypertension and prevents right heart failure via fatty acid oxidation,” published in…

Actelion Pharmaceuticals submitted a supplemental new drug application to U.S. regulatory authorities seeking expanded approval for Opsumit (macitentan) to treat inoperable chronic thromboembolic pulmonary hypertension (CTEPH). The U.S. Food and Drug Administration application is based on promising results from the Phase 2 MERIT-1 clinical trial (NCT02021292) that…

Signaling, or “crosstalk,” between smooth muscle and other cells in the lungs’ vasculature can promote the progression of pulmonary hypertension, research in mice shows. The findings point to mechanisms underlying PH that may lead to new treatments, especially ones targeting early stages of the disease. The research “Cell Autonomous…

Right ventricular function of the heart is the primary determining factor of survival in patients with pulmonary arterial hypertension (PAH) receiving specific treatments, a study suggests. The study, “Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study,” was published in the journal…

Surgery to repair congenital heart defects leads to longer survival in patients with pulmonary arterial hypertension (PAH) caused by heart disease, according to researchers. This finding was reported in the study, “Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts,” published…

Supplemental oxygen use is associated with improved survival in a subpopulation of patients with pulmonary arterial hypertension (PAH), a study has found. Current guidelines that recommend oxygen supplementation therapy for PAH patients are based on early data that showed the benefits of long-term oxygen therapy in patients with…

People with congenital heart disease are seven times more likely to develop pulmonary hypertension than the general population, a long-term Danish study indicates. In addition, congenital heart disease patients with PH are four times more likely to die than those without PH, the researchers reported. Their article, “Incidence and Mortality…

British researchers have identified rare mutations of four genes that could lead to pulmonary arterial hypertension. The team said scientists might be able to develop PAH treatments around the mutations of the genes GDF2, ATP13A3, AQP1, and SOX17. Their study, “Identification of rare sequence variation underlying heritable pulmonary…

University of Edinburgh researchers identifying a protein that could become a target for treating pulmonary arterial hypertension, or PAH. They discovered that the two-pore segment channel 2 protein, or TPC2, in pulmonary artery muscle cells regulates signals triggered by calcium that are important to controlling muscle cell contractions. The finding offers…

Arena Pharmaceuticals’ ralinepag reduced the risk of pulmonary arterial hypertension patients dying, a Phase 2 clinical trial showed. Temple University Professor Raymond Benza will present the results at the International Society for Heart and Lung Transplantation meeting in Nice, France, on April 14.  The four-day conference started April 11. The…

The genetic landscape of pulmonary arterial hypertension that strikes in childhood differs from the one that develops after a person reaches adulthood, a study reports. There is little variation between children and adults in gene mutations commonly associated with PAH, such as BMPR2. But children have more of the rarer…

A little-known government entity within the National Institutes of Health (NIH) is helping to lead U.S. efforts to speed up the development of therapies for some 7,000 rare diseases. The Office of Rare Diseases Research (ORDR), headquartered in Bethesda, Maryland, was established in 1993 within the NIH Office of the…