Impaired BMPR2 signalling across pulmonary hypertension subgroups: a multicentre study, European Respiratory Journal Open Research, September 28, 2026

A multicentre German study, run at specialist centres in Heidelberg, Hannover and Giessen/Marburg, looked at the key gene in pulmonary hypertension, BMPR2. Changes in this gene cause most inherited cases of pulmonary arterial hypertension, and the biological pathway it controls is also the target of sotatercept. The researchers took blood samples from 234 people in […]

Impaired BMPR2 signalling across pulmonary hypertension subgroups: a multicentre study, European Respiratory Journal Open Research, September 28, 2026 Read Post »

Alaunos Therapeutics has bought Velaris Therapeutics, which is developing four biological drugs for pulmonary arterial hypertension (PAH) and related heart and lung diseases, October 13, 2026

The pipeline VLR199 (the lead candidate): works in the same way as sotatercept (Winrevair), by trapping proteins of the activin family that drive the abnormal growth of cells in the lung vessels. The aim is to improve on aspects of sotatercept that may not be “fully optimised”. First clinical results showing whether it works are

Alaunos Therapeutics has bought Velaris Therapeutics, which is developing four biological drugs for pulmonary arterial hypertension (PAH) and related heart and lung diseases, October 13, 2026 Read Post »

What’s new on the Pulmonary Hypertension Knowledge Platform – September 2026

September was a busy month in the pulmonary hypertension world, with the European Society of Cardiology (ESC) and European Respiratory Society Congresses (ERS) bringing a wave of new research. The most important news is the update of the ERS Clinical Practice Guidelines for the treatment of pulmonary arterial hypertension (PAH), presented in Barcelona. AfPH Vice

What’s new on the Pulmonary Hypertension Knowledge Platform – September 2026 Read Post »

US Food and Drug Administration (FDA) updates Winrevair (sotatercept) label based on new trial data supporting earlier use in pulmonary arterial hypertension

The US Food and Drug Administration (FDA) has approved an update to the US label for WINREVAIR (sotatercept-csrk), Merck’s activin signaling inhibitor for pulmonary arterial hypertension, to include efficacy and safety data from the Phase 3 HYPERION trial. The trial specifically evaluated adults newly diagnosed with pulmonary arterial hypertension — within 12 months of screening,

US Food and Drug Administration (FDA) updates Winrevair (sotatercept) label based on new trial data supporting earlier use in pulmonary arterial hypertension Read Post »

Reduced brain blood flow, heart strain, and cognitive symptoms in pulmonary arterial hypertension, Therapeutic Advances in Respiratory Diseases, Jan-Dec 2026

Some patients with pulmonary arterial hypertension experience difficulties with memory, thinking, or mood. A group of Chinese researchers have investigated whether reduced blood flow to the brain is connected to heart strain and disease severity. They compared brain scans from 30 people with pulmonary arterial hypertension and 51 healthy individuals matched for age and sex.

Reduced brain blood flow, heart strain, and cognitive symptoms in pulmonary arterial hypertension, Therapeutic Advances in Respiratory Diseases, Jan-Dec 2026 Read Post »

Long-term efficacy and safety of rehabilitation in patients with pulmonary arterial hypertension: the FONCE-HTAP randomized controlled trial, Annals of the American Thoracic Society, July 20, 2026

The findings of a multi-center randomized controlled trial investigating the long-term efficiency of rehabilitation in patients with pulmonary arterial hypertension was published in the Annals of the American Thoracic Society on July 20, 2026. The study followed 47 patients with stable pulmonary arterial hypertension across 9 French centers, looking at the long-term impact of a

Long-term efficacy and safety of rehabilitation in patients with pulmonary arterial hypertension: the FONCE-HTAP randomized controlled trial, Annals of the American Thoracic Society, July 20, 2026 Read Post »

Predictive Power of Pulmonary Artery Stiffness – Right Ventricular Interplay, but not Pulmonary Vascular Resistance, in Mild Pulmonary Hypertension: A PVRI GoDeep meta-registry analysis, CHEST, August 26, 2026

In a recent LinkedIn post Prof. Werner Seeger writes that the GoDeep meta-registry analysis about the predictive power of pulmonary artery stiffness – right ventricular interplay in mild pulmonary hypertension (PH) is now online. He reports that in the analysis, notably, parameters integrating pulsatile vascular load and interdependent right ventricular function possess predictive power independent

Predictive Power of Pulmonary Artery Stiffness – Right Ventricular Interplay, but not Pulmonary Vascular Resistance, in Mild Pulmonary Hypertension: A PVRI GoDeep meta-registry analysis, CHEST, August 26, 2026 Read Post »

Multicentre study explores impaired bone morphogenetic protein receptor 2 (BMPR2) signalling across pulmonary hypertension subgroups, European Respiratory Journal Open Research 2026

A multicentre study recently published on the European Respiratory Journal Open Research looked at bone morphogenetic protein receptor 2 (BMPR2) signalling — a pathway already known to be disrupted in heritable pulmonary arterial hypertension (PAH) — across a wider range of pulmonary hypertension (PH) patients, to see whether the same disturbance shows up in other

Multicentre study explores impaired bone morphogenetic protein receptor 2 (BMPR2) signalling across pulmonary hypertension subgroups, European Respiratory Journal Open Research 2026 Read Post »

Insmed Announces Positive 12-Month Data from the Ongoing Open-Label Extension Study of Treprostinil Palmitil Inhalation Powder (TPIP) in Patients with Pulmonary Arterial Hypertension, July 16, 2026

Insmed announced on July 16 positive 12-Month data from the ongoing open-label extension (OLE) study evaluating treprostinil palmitil inhalation powder (TPIP), administered once daily in patients with pulmonary arterial hypertension (PAH, World Health Organization Group 1). The OLE study is a non-placebo-controlled trial and was designed to evaluate the long-term safety, tolerability, and effectiveness of

Insmed Announces Positive 12-Month Data from the Ongoing Open-Label Extension Study of Treprostinil Palmitil Inhalation Powder (TPIP) in Patients with Pulmonary Arterial Hypertension, July 16, 2026 Read Post »

Ralinepag for the treatment of pulmonary arterial hypertension (ADVANCE OUTCOMES): findings from the phase 3 study, The Lancet, July 26, 2026

ADVANCE OUTCOMES is a phase 3, randomised, double-blind trial tested oral ralinepag against placebo in pulmonary arterial hypertension patients, measuring time to first clinical worsening (death, hospitalisation, need for parenteral/inhaled prostacyclin therapy, disease progression, or unsatisfactory clinical response). Patients were enrolled between Jan 24, 2019, and June 20, 2025. Of 1037 patients screened for eligibility,

Ralinepag for the treatment of pulmonary arterial hypertension (ADVANCE OUTCOMES): findings from the phase 3 study, The Lancet, July 26, 2026 Read Post »

U.S. Food and Drug Administration (FDA) grants orphan drug status to IKT-001, an experimental formulation of imatinib to treat pulmonary arterial hypertension, July 23, 2026

The U.S. Food and Drug Administration (FDA) has granted orphan drug designation to IKT-001, an experimental formulation of imatinib that Inhibikase Therapeutics is developing to treat pulmonary arterial hypertension (PAH). Orphan drug designation is granted to investigational medications aimed at treating rare diseases — conditions affecting fewer than 200,000 people in the U.S. The designation is meant to give

U.S. Food and Drug Administration (FDA) grants orphan drug status to IKT-001, an experimental formulation of imatinib to treat pulmonary arterial hypertension, July 23, 2026 Read Post »

Study finds that early parenteral prostacyclin therapy enables near-normal haemodynamics in severe pulmonary arterial hypertension patients, European Respiratory Journal, July 16, 2026

In a single-centre Japanese cohort of 101 patients with haemodynamically severe pulmonary arterial hypertension treated with parenteral prostacyclin (1999–2023), 22 patients (22%) achieved near-normal haemodynamics and were successfully weaned off prostacyclin, all without adverse events, after a mean of 7.7 years of treatment. Those who de-escalated tended to have started prostacyclin earlier in their disease

Study finds that early parenteral prostacyclin therapy enables near-normal haemodynamics in severe pulmonary arterial hypertension patients, European Respiratory Journal, July 16, 2026 Read Post »

Study finds combining mindfulness with rehab exercise improves cardiopulmonary performance and psychological stress in pulmonary arterial hypertension patients, Canadian Respiratory Journal, June 29, 2026

Researchers at the Suqian First Hospital in Suqian, Chian, China tested whether adding mindfulness-based stress reduction (MBSR) to standard cardiopulmonary rehabilitation could benefit pulmonary arterial hypertension patients more than usual care alone. Fifty stable patients were split into two groups: one did a 6-week combined mindfulness-based stress reduction and rehab program, the other received standard

Study finds combining mindfulness with rehab exercise improves cardiopulmonary performance and psychological stress in pulmonary arterial hypertension patients, Canadian Respiratory Journal, June 29, 2026 Read Post »

Liquidia (LQDA) presents new YUTREPIA data at the US Pulmonary Hypertension Association (PHA)s International Annual conference in Dallas, June 11-14, 2026

Earlier this month, Liquidia Corporation presented four posters at the US Pulmonary Hypertension Association (PHA)’s International Conference in Dallas, sharing 24‑week ASCENT study data and broader insights on YUTREPIA (treprostinil) inhalation powder in pulmonary hypertension. Beyond clinical outcomes, the company highlighted patient‑reported cough data, the mental health strain on caregivers and the evolving role of

Liquidia (LQDA) presents new YUTREPIA data at the US Pulmonary Hypertension Association (PHA)s International Annual conference in Dallas, June 11-14, 2026 Read Post »

Corsair Pharma Announces Three Presentations of Phase 1 Clinical Data for TRX-248 Transdermal System at the 2026 Pulmonary Hypertension Association Conference

Corsair Pharma‘s experimental skin patch designed to deliver treprostinil, an approved treatment for pulmonary arterial hypertension (PAH), appears to be working as intended, according to newly announced data from an early clinical trial in healthy volunteers. Several treprostinil formulations are already approved for pulmonary arterial hypertension: subcutaneous 24/7 infusion (Remodulin and generics), oral (Orenitram, not

Corsair Pharma Announces Three Presentations of Phase 1 Clinical Data for TRX-248 Transdermal System at the 2026 Pulmonary Hypertension Association Conference Read Post »

Regeneron Pharmaceuticals expands Into pulmonary arterial hypertension with Phase 2 trial for REGN13335

Regeneron Pharmaceuticals, a U.S. biotech, is running a Phase 2 trial of REGN13335, an Anti-PDGF-B monoclonal antibody, in adults with pulmonary arterial hypertension who are already on standard treatment. It’s a randomized, placebo-controlled, quadruple-blind study (patients, doctors, investigators, and assessors are all kept in the dark during the main phase) to gauge what extra benefit

Regeneron Pharmaceuticals expands Into pulmonary arterial hypertension with Phase 2 trial for REGN13335 Read Post »

Upfront triple combination therapy with selexipag: insights from a real world cohort in Chinese patients with pulmonary arterial hypertension, Frontiers in Cardiovascular Medicine, May 21, 2026

This single-center retrospective study, conducted at Qilu Hospital, Shandong University (China), from February 2022 to November 2023, set out to compare two strategies for selexipag-based triple combination therapy in patients with pulmonary arterial hypertension (PAH): starting all three drugs together (initial/upfront triple therapy) versus adding selexipag on top of existing dual therapy (early sequential triple

Upfront triple combination therapy with selexipag: insights from a real world cohort in Chinese patients with pulmonary arterial hypertension, Frontiers in Cardiovascular Medicine, May 21, 2026 Read Post »

Haemodynamic Responses to Sotatercept and Parenteral Prostacyclins in Pulmonary Arterial Hypertension Patients, Journal of Heart and Lung Transplantation, June 1, 2026

French researchers retrospectively evaluated the haemodynamic trajectory of 62 patients with pulmonary arterial hypertension (PAH) who sequentially received parenteral prostacyclins and sotatercept as part of the French early access program. They found that both treatments lowered the resistance in the blood vessels of the lungs, making it easier for blood to flow. However, the treatments worked

Haemodynamic Responses to Sotatercept and Parenteral Prostacyclins in Pulmonary Arterial Hypertension Patients, Journal of Heart and Lung Transplantation, June 1, 2026 Read Post »

New study aims to see how triple combination therapy with sotatercept affects pulmonary vascular resistance in patient with newly diagnosed pulmonary arterial hypertension

New study aims to see how 24 weeks of triple therapy with an endothelin receptor antagonist (ERA), a phosphodiesterase-5 inhibitor (PDE5i), and sotatercept, affects pulmonary vascular resistance (PVR) in patients with newly diagnosed pulmonary arterial hypertension. SIRIUS is a 24-week, single-arm, open-label study with up to 42 days of screening and a 28-day safety follow-up.

New study aims to see how triple combination therapy with sotatercept affects pulmonary vascular resistance in patient with newly diagnosed pulmonary arterial hypertension Read Post »

United Therapeutics presents positive data from ongoing research at the American Thoracic Society’s International Conference, May 15-20, Orlando (US)

United Therapeutics announced positive data from the TETON-1 phase 3 pivotal study of inhaled treprostinil in idiopathic pulmonary fibrosis (IPF), and of the ADVANCE OUTCOMES and ARTISAN studies on pulmonary arterial hypertension at the American Thoracic Society’s International Conference on May 15-20, in Orlando (see also our previous article at this link) The following posters

United Therapeutics presents positive data from ongoing research at the American Thoracic Society’s International Conference, May 15-20, Orlando (US) Read Post »

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