Patricia Inácio, PhD, science writer —

Researchers found a new mutation in a gene — called eukaryotic translation initiation factor 2α kinase 4 (EIF2AK4)  — in members of a family with hereditary pulmonary arterial hypertension (HPAH). This mutation represents a potential “second hit” that increases the likelihood of certain family members developing the disease. The study, “…

Tyvaso (treprostinil sodium), a drug approved for enhancing exercise capacity in patients with pulmonary arterial hypertension (PAH), was reported in a recent study to be well tolerated and safe in routine clinical use. The study, “An observational study of inhaled-treprostinil respiratory-related safety in patients with pulmonary arterial hypertension,” was published…

Pulmonary arterial hypertension (PAH) severity is associated with alterations in the heart’s metabolism, but a recent study showed that Opsumit (macitentan) can reduce these metabolic changes, lessening PAH severity and easing strain on the heart’s right ventricle. The study, “Effects of an endothelin receptor antagonist, Macitentan, on right ventricular substrate utilization and function…

Sounds produced in the heart, reworked using a classification algorithm based on an automated speech recognition, were analyzed and seen to lead to more accurate diagnoses of pulmonary hypertension (PH) in patients than those done by physicians listening to the same heart recordings, according to the study,  “Acoustic diagnosis of pulmonary hypertension: automated…

During their development, babies who will be born with Down syndrome have higher levels of anti-angiogenic factors in their lungs, impairing fetal lung vessel growth and potentially contributing to their developing pulmonary arterial hypertension (PAH), researchers report. The study, “Increased Lung Expression of Anti-Angiogenic Factors in Down Syndrome: Potential Role in…

People with pulmonary arterial  hypertension (PAH) have higher than usual levels of a protein produced in cells in response to shock or stress — called heat shock protein 90 or HSP90 — and diminishing those levels helped to prevent pulmonary hypertension from progressing in a rat model of the disease, researchers reported.

Low-risk pulmonary arterial hypertension (PAH) patients may be able to switch from pump-infusion parenteral prostacyclin therapy to more convenient oral therapies, according to the study, “Transition from parenteral to oral treprostinil in pulmonary arterial hypertension,” published in the Journal of Heart and Lung Transplantation. Prostanoids, which include prostaglandins, thromboxanes and prostacyclins,…

A prospective analysis of two clinical trials, PATENT-1 and PATENT-2, showed that riociguat (Adempas) treatment improves several parameters of lung and cardiac function in patients with pulmonary arterial hypertension (PAH), and in those with PAH associated with connective tissue disease (PAH-CTD). The study “Riociguat for the treatment of pulmonary arterial hypertension…

The delivery and efficiency of ethyl pyruvate (EP), an anti-inflammatory and anti-oxidant agent, was significantly improved in a rat model of pulmonary arterial hypertension (PAH) when encapsulated in nanoparticles (NPs). The study, “Intratracheal instillation of ethyl pyruvate nanoparticles prevents the development of shunt-flow-induced pulmonary arterial hypertension in a rat…

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…

Riociguat treatment administered at steady state (2.5 mg, three times a day) to women with pulmonary arterial hypertension (PAH) did not alter the effectiveness of oral contraceptives or make their use unsafe, researchers reported. The study, “Pharmacokinetic interaction study between riociguat and the combined oral contraceptives levonorgestrel and ethinylestradiol in…

A microRNA, called miR-214, promotes excessive cellular proliferation in pulmonary arterial hypertension (PAH), and decreasing its expression may significantly impair phenotype changes in the lung vasculature in PAH, researcher report.The study, “MEF2C-MYOCD and Leiomodin1 Suppression by miRNA-214 Promotes Smooth Muscle Cell Phenotype Switching in Pulmonary Arterial Hypertension,” was published…