PAH News

The blood levels of two molecules involved in vascular remodeling, a hallmark feature of pulmonary arterial hypertension (PAH), may be used as noninvasive predictive biomarkers of the disease, a small study suggests. The study, “Fibrinolytic markers could be useful predictors of severity in patients with pulmonary…

Adults with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) who have trouble breathing at rest or with light activity may benefit from a procedure called respiratory rate modulation, a pilot, single-center study shows. The three-month procedure also led to lower symptom severity and decreased respiratory…

Bilateral lung transplant rapidly and effectively normalizes right ventricular function of the heart in children with severe pulmonary arterial hypertension (PAH) and heart failure, a study reports. These benefits in the 15 children evaluated were also independent of a child’s age, weight, and right ventricular (RV) impairment before the transplant. Given…

The U.S. Food and Drug Administration (FDA) has granted tentative approval to Yutrepia (treprostinil), previously known as LIQ861, as an inhalation treatment for pulmonary arterial hypertension (PAH), its developer Liquidia announced. Tentative approval means that Yutrepia has met all regulatory standards regarding quality, safety, and efficacy required for approval in the U.S. Because…

Potential targets to treat pulmonary arterial hypertension (PAH) were identified by analyzing the gene activity of individual lung endothelial cells isolated from a mouse model of the disease. Study results were validated by cross-referencing them with rat and human gene activity datasets. “The identification of distinct molecular mechanisms and…

Children undergoing high-dose chemotherapy and hematopoietic stem cell transplant (HSCT) to treat their cancer should be screened for pulmonary arterial hypertension (PAH), according to a case report of two young patients at a Pennsylvania hospital. Its authors noted that other reports also suggested “possible links” between such treatments and PAH…

Due to problems at a third-party facility, the U.S. Food and Drug Administration (FDA) has decided to not approve Tyvaso DPI, a dry powder inhaled formulation of treprostinil, at this time. According to United Therapeutics, the medication’s developer, the issue is expected to be dealt with soon. The company anticipates…

The U.S. Food and Drug Administration (FDA) has cleared Tenax Therapeutics to begin clinical testing of its new oral formulation of imatinib in people with pulmonary arterial hypertension (PAH). The company is planning to conduct a small pharmacological study this year — and then launch a Phase 3…

Infusion with Ferinject (ferric carboxymaltose) safely treated people with pulmonary arterial hypertension (PAH) and iron deficiency, easing disease symptoms, improving exercise capacity, and lowering their number of hospital visits over a period of 18 months, a long-term study reported. Ferinject, as this treatment for iron deficiency anemia is known…

Blocking the activity of a small RNA molecule called microRNA-30a eased alterations in heart structure and blood vessel architecture in mouse models of pulmonary arterial hypertension (PAH), a new study reports. Results also showed that this molecule is present at abnormally high levels in PAH patients, supporting its potential…

Rare mutations in the ATP13A3 gene caused a severe inherited form of childhood-onset pulmonary arterial hypertension (PAH), a case study of three families showed. These findings demonstrate the “growing importance” of genetic testing in patients with childhood-onset PAH, the scientists said, noting that the genetic basis of the disease…

Health-related quality of life does not change substantially over time among people with pulmonary arterial hypertension (PAH) — though PAH patients who experience more substantial disease progression tend to report worse quality of life scores — a small study done at a hospital in France has found. These findings…