Lindsey Shapiro, PhD,  science writer—

AV-101, an inhaled formulation of imatinib for people with pulmonary arterial hypertension (PAH) now being tested in clinical trials, was found in a series of experiments in animal models to result in better lung exposure than oral formulations of the medication. By offering direct delivery to the lungs, AV-101’s…

A gene called RASA3 may be a candidate gene — one suspected of playing a role in a specific trait or disorder — in the development of pulmonary hypertension (PH) among people with sickle cell disease (SCD), according to a new study. Reduced activity of RASA3 was evident…

Noninvasive clinical assessments to identify children with pulmonary arterial hypertension (PAH) at risk of poorer outcomes align well with right heart catheterization (RHC), an invasive procedure, but they are not accurate enough to forgo a need for RHC altogether, according to a recent study. Scientists found that about 70%…

Team PHenomenal Hope — Team PH — a nonprofit organization seeking to raise awareness about pulmonary hypertension (PH), has launched an educational website for people newly diagnosed or who are living with the rare disease. The site, called Learn Live Breathe PH, was launched Feb. 28 in…

Pulmonary endarterectomy (PEA) and balloon pulmonary angioplasty (BPA) — two treatment approaches for chronic thromboembolic pulmonary hypertension (CTEPH) — significantly improved blood flow dynamics and functional capacity in patients given either procedure, a study from Norway reported. PEA was more effective at reducing pressure and resistance in the lungs’…

Note: The headline of this article was updated Feb. 27, 2023, to better reflect that Veletri is considered a safe and effective therapy at the approved and recommended dosing levels.  Treatment with Veletri (epoprostenol AS) infusion was generally well-tolerated and safe, but did not lead to clinical improvements for…

People with pulmonary hypertension (PH) who had elevated blood levels of the trimethylamine N-oxide (TMAO) molecule were at a five times greater risk of a poor prognosis than patients who didn’t have high levels, according to a recent study. High levels of this gut-derived metabolite were generally linked to…

Dosing is complete in a Phase 1 clinical trial of AER-901, Aerami Therapeutics’ inhaled formulation of imatinib that’s being investigated to treat pulmonary arterial hypertension (PAH). Aerami plans to advance the experimental therapy to a proof-of-concept Phase 2 trial in the first half of 2023. To do so,…

Researchers have created an automated way to engineer a more stable peptide-based therapy, which could inform the production of new and improved treatments for diseases like pulmonary arterial hypertension (PAH). The research was led by Yousef Al-Abed, PhD, co-director of the Institute of Bioelectronic Medicine at the Feinstein Institutes for…

Real-world use of Opsumit (macitentan) was generally safe, with few liver-related toxicities seen, according to data from two registries largely made up of pulmonary arterial hypertension (PAH) patients. Collective data from the two registries met a requirement from the U.S. Food and Drug Administration (FDA) for additional real-world safety…

A rare mutation in the GDF2 gene was identified in two siblings, a boy and a girl, with pulmonary arterial hypertension (PAH), according to a recent case report. The mutation, which each child inherited in both gene copies, resulted in a lack of the bone morphogenetic protein (BMP) 9 in the…

People with pulmonary arterial hypertension (PAH) are at a higher risk of acute heart failure when admitted to the hospital for sepsis or septic shock than are patients without PAH, a U.S. study found. Sepsis — the body’s severe response to infection — in PAH patients, however, was not…