Pulmonary arterial hypertension associated with exposure to toxic rapeseed oil, European Respiratory Journal Open Research, December 19, 2025

A prospective analysis from the Spanish Registry of pulmonary arterial hypertension (REHAP) examined 59 patients diagnosed with toxic oil syndrome-associated pulmonary arterial hypertension (TOS-PAH) between 1997 and 2025, providing new clinical, pathological and genetic insights into this historically significant form of drug-induced pulmonary arterial hypertension. Key findings: The patient cohort was 63% female with a […]

Pulmonary arterial hypertension associated with exposure to toxic rapeseed oil, European Respiratory Journal Open Research, December 19, 2025 Read Post »

The acute pulmonary vasoreactivity in pulmonary arterial hypertension revisited in the contemporary era, International Journal of Cardiology Heart and Vasculature, February 2026

During right heart catheterization (RHC), the “acute vasodilator test” offers valuable insights into pulmonary circulation by evaluating vasoreactivity. A positive result identifies a rare pulmonary arterial subtype that may benefit from long-term calcium-channel blocker therapy (the so called “responders”). A recent study re-examines the applications, criteria, and objectives of acute vasodilator testing in pulmonary hypertension

The acute pulmonary vasoreactivity in pulmonary arterial hypertension revisited in the contemporary era, International Journal of Cardiology Heart and Vasculature, February 2026 Read Post »

Understanding the Connection Between Pulmonary Hypertension & Scleroderma, live webinar by the Canadian Pulmonary Hypertension Association PHA Canada, January 13, 2025

Join Dr. Nathan Hambly, a respirologist at the Firestone Institute for Respiratory Health at St. Joseph’s Healthcare, Hamilton, Canada, as he explores why pulmonary arterial hypertension happens in scleroderma, how to spot the signs early, and what care options exist today. 🗓️ January 13 | 🕐 1:30 pm EST | 💻 Online🎙️ Hosted in partnership

Understanding the Connection Between Pulmonary Hypertension & Scleroderma, live webinar by the Canadian Pulmonary Hypertension Association PHA Canada, January 13, 2025 Read Post »

Durability of sotatercept response in pulmonary arterial hypertension: Insights from extended real-world follow-up, International Journal of Cardiology, January 2, 2026

A retrospective cohort study using data from 103 healthcare organizations compared 454 pulmonary arterial hypertension patients receiving sotatercept to 963.386 pulmonary arterial hypertension patients not receiving sotatercept. After propensity score matching to balance baseline characteristics, 346 patients per group were analyzed over an average follow-up of approximately 6-8 months. The sotatercept group demonstrated dramatically lower

Durability of sotatercept response in pulmonary arterial hypertension: Insights from extended real-world follow-up, International Journal of Cardiology, January 2, 2026 Read Post »

Inserm, France’s National Institute of Health and Medical Research, awards its 2025 Grand Prix to Prof. Marc Humbert for his role in transforming pulmonary hypertension care

Professor Marc Humbert, one of the world’s leading specialists in pulmonary arterial hypertension (PAH), has been awarded the prestigious Inserm Grand Prize in recognition of his extraordinary career dedicated to understanding and treating this life-threatening disease. Inserm is the French National Institute of Health and Medical Research. From Orphan Disease to Hope When Prof. Humbert

Inserm, France’s National Institute of Health and Medical Research, awards its 2025 Grand Prix to Prof. Marc Humbert for his role in transforming pulmonary hypertension care Read Post »

The Pulmonary Vascular Research Institute (PVRI) 2026 Congress is taking place in Dublin from 28 January-1 February 2026

The Pulmonary Vascular Research Institute (PVRI) Congress 2026 will take place in the historic city of Dublin from 28 January – 1 February 2026. ➡️ Explore the full Congress programme at this link The first and last day (28 January and 1 February) will be satellite meetings with the main plenary sessions happening 29-31 January.

The Pulmonary Vascular Research Institute (PVRI) 2026 Congress is taking place in Dublin from 28 January-1 February 2026 Read Post »

The European Reference Network for Rare Lung Diseases (ERN Lung) to implement universal online Patient-Reported Outcome Measure, ERN-Lung Newsletter, Issue 41, December 2025

Patient-reported outcome measures (PROMs) capture patients’ own perspectives on their health and are crucial for improving care, yet they remain underused in clinical practice due to barriers like time constraints, language availability, and licensing costs. The European Reference Center for Rare Lung Diseases (ERN LUNG) is launching an ambitious initiative to implement a standardized PROM

The European Reference Network for Rare Lung Diseases (ERN Lung) to implement universal online Patient-Reported Outcome Measure, ERN-Lung Newsletter, Issue 41, December 2025 Read Post »

Male survival disadvantage in pulmonary hypertension: independent of aetiology, age, disease severity, comorbidities and treatment, EBioMedicine, December 16, 2025

The Pulmonary Vascular Research Institute (PVRI) GoDeep meta-registry integrates data from international pulmonary hypertension registries, from which 21,123 incident hemodynamically fully characterised patients were analysed to investigate the influence of sex on survival of patients. Findings revealed that male patients have significantly higher mortality rates than female patients across all types of pulmonary hypertension. This survival

Male survival disadvantage in pulmonary hypertension: independent of aetiology, age, disease severity, comorbidities and treatment, EBioMedicine, December 16, 2025 Read Post »

The Discovery of Inhaled Prostanoids for Pulmonary Hypertension, Prof. Werner Seeger, Pulmonary Circulation, December 18, 2025

A very interesting article by Prof. Werner Seeger recounts the discovery and development of inhaled prostanoids for pulmonary hypertension at the Giessen Pulmonary Hypertension center (Germany) in the late 1980s-1990s (§). Initially researching acute lung injury/Acute Respiratory Distress Syndrome (ARDS), researchers at Giessen observed that while intravenous vasodilators worsened ventilation/perfusion mismatch, aerosolized prostacyclin (PGI2) reduced

The Discovery of Inhaled Prostanoids for Pulmonary Hypertension, Prof. Werner Seeger, Pulmonary Circulation, December 18, 2025 Read Post »

Use of Sotatercept to Facilitate Transition From Intravenous to Oral Prostacyclin Therapy for Pulmonary Arterial Hypertension, Clinical Respiratory Journal, December 19, 2025

Parenteral prostacyclin therapy remains the cornerstone for high-risk pulmonary arterial hypertension (PAH) patients but is limited by complications and reduced quality of life. A case series of eight high-risk patients demonstrated that adding sotatercept successfully enabled transition from intravenous to oral prostacyclin therapy. All patients received background dual therapy with endothelin receptor antagonists and phosphodiesterase-5

Use of Sotatercept to Facilitate Transition From Intravenous to Oral Prostacyclin Therapy for Pulmonary Arterial Hypertension, Clinical Respiratory Journal, December 19, 2025 Read Post »

ASPYRE-1 study: An Italian multicenter prospective study on pulmonary hypertension modality of death and validation of reveal risk score, Vascular Pharmacology, November 29, 2025

The ASPYRE-1 study presents the baseline characteristics of patients enrolled in the national prospective registry created through the Italian Network on Pulmonary Arterial Hypertension (IPHNET). The registry was established in 2014 with the aim of better understanding the contemporary profile of Italian pulmonary arterial hypertension patients and to support future analyses on risk stratification and

ASPYRE-1 study: An Italian multicenter prospective study on pulmonary hypertension modality of death and validation of reveal risk score, Vascular Pharmacology, November 29, 2025 Read Post »

Patient-reported Functional Class (FC) may better reflect pulmonary hypertension burden than physician-assigned Functional Class, CHEST, November 27, 2025

A new study published recently on CHEST evaluated whether the Pulmonary Hypertension Functional Class Self-Report (PH-FC-SR)—a patient-reported version of the World Health Organisation (WHO) Functional Class—captures functional status in a way that reflects patients’ real experiences. Study Overview Eighty-nine adults with pulmonary hypertension (Groups 1–5) from the Cleveland Clinic and Mayo Clinic completed an online

Patient-reported Functional Class (FC) may better reflect pulmonary hypertension burden than physician-assigned Functional Class, CHEST, November 27, 2025 Read Post »

Efficacy of 10% lidocaine gel for injection site pain associated with treprostinil in the treatment of pulmonary hypertension: a report of four cases, JA Clinical Reports, November 29, 2025

This is a small study but it addresses a very important but often overlooked issue, that of injection site pain for pulmonary arterial hypertension patients being treated with sub-cutaneous treprostinil, which can significantly affect quality of life. The researchers found that topical 10% lidocaine gel provided rapid and effective relief of injection site pain, facilitating

Efficacy of 10% lidocaine gel for injection site pain associated with treprostinil in the treatment of pulmonary hypertension: a report of four cases, JA Clinical Reports, November 29, 2025 Read Post »

A comprehensive review of drugs and toxins associated with pulmonary arterial hypertension offers clinicians updated guidance, European Respiratory Journal Open Research, November 2025 

A review recently published in the European Respiratory Journal Open Research provides the latest evidence on drug- and toxin-associated pulmonary arterial hypertension, offering clinicians updated guidance on recognition, diagnosis, and management of this pulmonary arterial hypertension subtype (see below for the most recent pulmonary arterial hypertension classification, at 1.3) The first World Health Organization (WHO)

A comprehensive review of drugs and toxins associated with pulmonary arterial hypertension offers clinicians updated guidance, European Respiratory Journal Open Research, November 2025  Read Post »

Why Wait for Sick People to Get Sicker? The Paradox of the Treatment of Patients With Pulmonary Arterial Hypertension, Pulmonary Circulation, November 20, 2025

Pulmonary arterial hypertension (PAH) is a progressive, life-threatening disease where early detection is strongly encouraged to prevent irreversible vascular and cardiac damage. However, a fundamental paradox exists: while clinicians are urged to diagnose pulmonary arterial hypertension early, treatment is restricted to symptomatic patients (New York Heart Association (NYHA) functional class II or higher), leaving asymptomatic

Why Wait for Sick People to Get Sicker? The Paradox of the Treatment of Patients With Pulmonary Arterial Hypertension, Pulmonary Circulation, November 20, 2025 Read Post »

GDF15 is a promising biomarker for distinguishing pulmonary veno-occlusive disease (PVOD) and pulmonary arterial hypertension (PAH), Journal of Clinical Investigation, November 20, 2025

Pulmonary veno-occlusive disease (PVOD) is a rare, severe World Health Organisation Group 1 pulmonary arterial hypertension (PAH) subtype with poor survival. pulmonary arterial hypertension-targeted vasodilators can cause life-threatening pulmonary edema in Pulmonary veno-occlusive disease, underscoring the need for diagnostic tools to distinguish it from other pulmonary arterial hypertension subtypes The findings of a study recently

GDF15 is a promising biomarker for distinguishing pulmonary veno-occlusive disease (PVOD) and pulmonary arterial hypertension (PAH), Journal of Clinical Investigation, November 20, 2025 Read Post »

Evolving paradigms in pulmonary hypertension: Highlights of the 6th French Pulmonary Hypertension Network Meeting, Respiratory Medicine and Research, Vol 88, November 2025

The 6th “Journées du Réseau Français de l’Hypertension Pulmonaire” (JRF-HTP), held in October 2024, provided a review of the evolving landscape in pulmonary hypertension (PH). The meeting brought together clinicians and researchers to exchange on the most recent scientific, clinical, and organizational advances in pulmonary hypertension. We are proud that a patient advocate was included as co-author,

Evolving paradigms in pulmonary hypertension: Highlights of the 6th French Pulmonary Hypertension Network Meeting, Respiratory Medicine and Research, Vol 88, November 2025 Read Post »

Treatment options for children with pulmonary arterial hypertension associated with congenital heart disease (PH-CHD), Expert Review of Respiratory Medicine, October 30, 2025

Pulmonary hypertension associated with congenital heart disease (PAH-CHD) is one of the most common causes of pulmonary hypertension in children. Patients fall into different subgroups, each requiring specific treatment approaches. According to an article recently published in the Expert Review of Respiratory Medicine current management is clear for two groups: The challenge The management of

Treatment options for children with pulmonary arterial hypertension associated with congenital heart disease (PH-CHD), Expert Review of Respiratory Medicine, October 30, 2025 Read Post »

Lung Transplant Allocation System Challenges for Pulmonary Arterial Hypertension (PAH) Patients, Letter to the Editor, Transplant International, October 28, 2025

Pulmonary arterial hypertension patients face unique challenges in lung transplantation. Unlike patients with other lung diseases, they often have relatively normal lung function tests but are in critical condition due to heart failure. This means they receive low priority scores in the standard Lung Allocation Score (LAS) system, which was designed primarily for patients with

Lung Transplant Allocation System Challenges for Pulmonary Arterial Hypertension (PAH) Patients, Letter to the Editor, Transplant International, October 28, 2025 Read Post »

Comparing Pulmonary Arterial Hypertension Care in Urban and Rural Settings: Treatment Patterns and Risk Trajectories, Pulmonary Circulation, November 11, 2025

Access to specialized care may differ between urban and rural patients, potentially influencing outcomes. A study conducted at the at the University of Utah Pulmonary Hypertension Center compared 263 pulmonary arterial hypertension patients from urban versus rural areas treated in the period 2020-2024. Similar at baseline: Important differences emerged: Conclusion: Despite similar care delivery and

Comparing Pulmonary Arterial Hypertension Care in Urban and Rural Settings: Treatment Patterns and Risk Trajectories, Pulmonary Circulation, November 11, 2025 Read Post »

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