A drug approved for a genetic condition called urea cycle disorder — sodium 4-phenylbutyric acid (4-PBA) — shows a protective effect for pulmonary arterial hypertension (PAH) by decreasing pulmonary artery pressure and mean right ventricular pressure in a PAH rat model. The study, “4-Phenylbutyric Acid Induces Protection against Pulmonary Arterial Hypertension in…
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Reata Pharmaceuticals has reported positive interim results from the extension Phase 2 LARIAT clinical trial assessing bardoxolone methyl for the treatment of pulmonary arterial hypertension (PAH). The LARIAT trial is assessing the efficacy, safety, and tolerability of bardoxolone methyl compared to a placebo in patients with PAH. The study aims to determine the recommended…
Researchers from the U.S., Germany, Switzerland, and the U.K. have developed a new questionnaire, to help assess symptoms of pulmonary arterial hypertension (PAH) and the disease’s impact on patients’ lives. The questionnaire, called PAH-SYMPACT, was created following guidance from the U.S. Food and Drug Administration (FDA) to include patient reported outcomes…
Scientists discovered that levels of a blood protein called hematoma derived growth factor (HDGF) increases as pulmonary arterial hypertension (PAH) becomes more severe, which now raises belief that a noninvasive test for the protein could shorten the path to PAH diagnosis and allow treatment at earlier disease stages. The discovery by …
Recently published data from the CHEST-2 clinical trial showed that riociguat (Adempas) is both safe and effective as a longer term treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The study also identified two predictive biomarkers distinguishing CTEPH and pulmonary arterial hypertension (PAH), and their concentrations serve as prognostic guides of…
SteadyMed, Ltd., and the Pulmonary Hypertension Association (PHA) recently launched a new campaign, called PHenomenal, offering patients with pulmonary hypertension (PH) and their loved ones a social media outlet through which to share photos and videos that highlight how fascinating and inspirational they are as people. “My patients with PH continually impress me…
Bellerophon Therapeutics, Inc., announced the enrollment of the first patient with pulmonary arterial hypertension (PAH) in its Phase 3 INOvation-1 clinical study, assessing the efficacy and safety of the next-generation INOpulse delivery system. The portable device is designed to continuously administer inhaled nitric oxide (iNO) to ambulatory patients using a carefully controlled,…
Actelion recently announced that its oral drug Uptravi (selexipag) is now commercially available in Germany for the treatment of pulmonary arterial hypertension (PAH). “The availability of Uptravi is a milestone for PAH patients in Germany, where until now, the options for treatments targeting the prostacyclin pathway have been limited, and were burdensome…
A new study from investigators in Wisconsin suggests that treatments reducing the production and breakdown of collagen may be used as therapies for pulmonary arterial hypertension (PAH). The research report “Limiting collagen turnover via collagenase‐resistance attenuates right ventricular dysfunction and fibrosis in pulmonary arterial hypertension,” was published in the online journal Physiological Reports.
Researchers at the Nagoya University Graduate School of Medicine in Japan demonstrated that the endothelin receptor antagonist bosentan can improve endothelial dysfunction parameters in patients with pulmonary arterial hypertension (PAH), but not in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). The findings were published in the Pulmonary…
Researchers from Tufts Medical Center in Boston identified two new biomarkers that may help predict survival in patients with pulmonary arterial hypertension (PAH). Findings from the study “Plasma 12- and 15-hydroxyeicosanoids are predictors of survival in pulmonary arterial hypertension,” were recently published in the journal Pulmonary Circulation. PAH…
Mutations in the ENG gene might contribute to disease mechanisms in pulmonary arterial hypertension (PAH), according to a recent study demonstrating that PAH patients often carry ENG gene changes in addition to other, more well-studied mutations. The study demonstrates that genetic contributions to PAH are more complex than anticipated, raising the…
