News

Combined therapy with Revatio (sildenafil) and Opsumit (macitentan) was effective in a patient diagnosed with pulmonary hypertension (PH) associated with a rare blood disorder called polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, a case report shows. The…

Increased levels of a protein called LRP1 may lead to blood vessel remodeling in pulmonary hypertension (PH) by causing changes in pulmonary artery cells that favor their proliferation, a study suggests. The study, “LRP1 promotes synthetic phenotype of pulmonary artery smooth muscle cells in pulmonary hypertension,”…

Systemic hypertension is an independent predictor in rare cases of pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE), a retrospective large-cohort study in Taiwan shows. According to the findings, most of the observed PAH cases occurred within five years of SLE diagnosis. The study, “Incidence and…

Variations in a gene called FIGN predispose to familial or heritable pulmonary arterial hypertension (FPAH) in carriers of the p.Arg491Gln mutation at the BMPR2 gene, according to a study looking at the largest family known to date with a history of the disease. The findings may help to advance understanding…

Increased production of a protein called Nestin by cells lining the blood vessels in the lungs may contribute to the development and progression of pulmonary arterial hypertension (PAH), a study suggests. Additional studies are still warranted to further understand whether targeting Nestin might represent a viable and effective approach…

Patients with sarcoidosis-associated pulmonary hypertension (SAPH) can improve their levels of a key biomarker after receiving specific treatment for PH, which suggests that PH-specific treatment may benefit this patient population, according to a real-world study. The study, “Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary…

The pharmaceutical company Sandoz announced the commercialization of an injectable form of the vasodilating therapy treprostinil for treating exercise-related symptoms affecting individuals with pulmonary arterial hypertension (PAH). This treprostinil injection is the first generic substitute (the non-branded version of a developed therapy) of Remodulin, developed and sold…

Note: This is the first part of a three-part series written in collaboration with respiratory therapist Mark W. Mangus Sr., RRT, RPFT, FAARC, and oxygen expert Ryan Diesem. One of the more hotly debated topics in the treatment of respiratory disease is supplemental oxygen use. Participants in the debate often…

Mutations in the BMPR2 gene alter the ratio of pro- and anti-death signals in cells lining the blood vessels, promoting cells destined to die to escape, which in turn culminates in a build-up of cells in blood vessels and their subsequent blockage, leading to pulmonary arterial hypertension (PAH). The factor mediating this resistance to…

Heart defects that develop before birth may lead to a variety of complications later in life, including pulmonary hypertension (PH), according to cardiologist Sanjay Gandhi, MD. However, he says, it is still not possible to predict if or when these complications will arise. Congenital heart defects (CHDs) occur when the…

People with polysplenia syndrome — a condition characterized by a “left-sidedness” on both sides of the body — have an increased risk for early development of pulmonary hypertension (PH), a Japanese study suggests. The study, titled “Polysplenia Syndrome as a Risk Factor for Early Progression of Pulmonary Hypertension,” was…

Resverlogix announced that it has received funding from the Canadian Institutes for Health Research for a Phase 2 clinical study investigating the safety and efficacy of its proprietary therapy apabetalone (RVX-208) as a potential treatment for pulmonary arterial hypertension (PAH). Resverlogix will match the new funding, including in-kind…