How it all started: The Aminorex Epidemic that Launched Systematic Primary Pulmonary Hypertension (PPH) Studies and the First Patient Registry

An article titled “The Genesis and Legacy of the NHLBI Patient Registry for the Characterization of Primary Pulmonary Hypertension”, published recently in Pulmonary Circulation, traces the historical development of primary pulmonary hypertension (PPH) research from its first pathological description by Ernest Romberg in 1891 through the establishment of the landmark US National Heart, Lung, and […]

How it all started: The Aminorex Epidemic that Launched Systematic Primary Pulmonary Hypertension (PPH) Studies and the First Patient Registry Read Post »

What can ERN-LUNG offer for the future? Special session at the European Respiratory Society Annual Congress, Amsterdam, October 1, 2025 – 09:30–11:30 CEST

On Wednesday October 1, 2025, a special programme at the European Respiratory Congress will be discussing what can ERN-LUNG offer for the future. ERN-LUNG is a patient-centric network of European healthcare providers and patient organisations, committed Europe-wide and globally to reducing morbidity and mortality from rare lung diseases in people of all ages through: ✅

What can ERN-LUNG offer for the future? Special session at the European Respiratory Society Annual Congress, Amsterdam, October 1, 2025 – 09:30–11:30 CEST Read Post »

The causal effect of gut microbiome on pulmonary artery hypertension based on a two-sample Mendelian randomization study, Nutrition & Metabolism, July 29, 2025

Previous studies have shown that pulmonary arterial hypertension patients have different gut bacteria compositions, with lower diversity and fewer anti-inflammatory bacteria that produce beneficial short-chain fatty acids. Could the gut-lung connection contribute to pulmonary arterial hypertension development through enhanced nerve communication and altered bacterial metabolites like trimethylamine-N-oxide? In this two-sample Mendelian randomization study, recently published

The causal effect of gut microbiome on pulmonary artery hypertension based on a two-sample Mendelian randomization study, Nutrition & Metabolism, July 29, 2025 Read Post »

Real-life use of quality of life questionnaires in pulmonary arterial hypertension in Spain, European Journal of Cardiovascular Nursing, July 7, 2025

Despite strong recommendations to assess quality of life in pulmonary arterial hypertension patients from the World Symposia on Pulmonary Hypertension (WSPH) in 2018 and 2024, and from the 2022 European Society of Cardiology (ESC) and European Respiratory Society (ERS) joint pulmonary hypertension guidelines, this evaluation remains rarely implemented in clinical practice. This cross-sectional study of

Real-life use of quality of life questionnaires in pulmonary arterial hypertension in Spain, European Journal of Cardiovascular Nursing, July 7, 2025 Read Post »

Pulmonary endarterectomy vs balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension, BMC Pulmonary Medicine, August 6, 2025

A study recently published on BMC Pulmonary Medicine compared baseline characteristics and short-term efficacy of pulmonary endarterectomy (gold standard for operable chronic thromboembolic pulmonary hypertension, removing thickened intima and organized thrombus from pulmonary arteries) vs. balloon pulmonary angioplasty (an option for inoperable chronic thromboembolic pulmonary hypertension, improving blood flow by dilating stenosed/occluded vessels without removing lesions), particularly

Pulmonary endarterectomy vs balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension, BMC Pulmonary Medicine, August 6, 2025 Read Post »

Risk Assessment Models and Event‐Free Survival in Pulmonary Arterial Hypertension, Pulmonary Circulation, July 18, 2025

The Research: Evidence on the predictive ability of risk assessment models for event-free survival (EFS) in patients with pulmonary arterial hypertension is scarce, say the authors of a recent study published on Pulmonary Circulation. The aim of the study was to evaluate how well three different risk assessment models predict event-free survival. The study included 411

Risk Assessment Models and Event‐Free Survival in Pulmonary Arterial Hypertension, Pulmonary Circulation, July 18, 2025 Read Post »

Pulmonary Artery Denervation for Idiopathic Pulmonary Arterial Hypertension: A Case Report, Catheterization & Cardiovascular Interventions (CCI), July 29, 2025

Current targeted medications for pulmonary arterial hypertension can help symptoms but have limited long-term effectiveness and often cause significant side effects and high costs. Percutaneous pulmonary artery denervation is a realtively new interventional procedure that selectively destroys sympathetic nerves to restore nerve and hormone balance in patients who don’t respond well to standard medications – see

Pulmonary Artery Denervation for Idiopathic Pulmonary Arterial Hypertension: A Case Report, Catheterization & Cardiovascular Interventions (CCI), July 29, 2025 Read Post »

Inflammation and Weight in Pulmonary Hypertension – Surprising Differences in Outcomes, American Journal of Respiratory and Critical Care Medicine, August 6, 2025

A recent study titled “Inflammation and Obesity Correlate in Pulmonary Hypertension but Associate with Diverging Outcomes” analysed two common health factors in people with pulmonary hypertension: inflammation and body weight. Inflammation is associated with all types of pulmonary hypertension. In the study inflammation was measured by a blood test called C-reactive protein (CRP), and body

Inflammation and Weight in Pulmonary Hypertension – Surprising Differences in Outcomes, American Journal of Respiratory and Critical Care Medicine, August 6, 2025 Read Post »

From Ancient Mysteries to Modern Miracles: The Incredible Journey of Pulmonary Circulation, Adam Torbicki, “Diagnostics”

INTRODUCTION Ever wondered how we went from completely misunderstanding blood flow for 1,400 years to performing open-heart surgery? A fascinating historical review by Prof. Adam Torbicki, published in Diagnostics (2021), reminds us that while most modern clinicians “look forward, expecting new pathophysiological pathways to be explored,” few take time “to look into the past.” Yet

From Ancient Mysteries to Modern Miracles: The Incredible Journey of Pulmonary Circulation, Adam Torbicki, “Diagnostics” Read Post »

Important announcement: the date for the next World Symposium on Pulmonary Hypertension is now set!

Traditionally the World Pulmonary Hypertension Symposia series, started in Geneva in 1973 and held every 5 years after the Evian edition in 1998 (Venice 2003, Dana Point 2008, Nice 2013, Nice 2018), has marked the progresses in pulmonary hypertension science and has anticipated future developments. The published supplement including the symposia proceedings constitutes a collection

Important announcement: the date for the next World Symposium on Pulmonary Hypertension is now set! Read Post »

New study finds possible diagnostic biomarkers for idiopathic pulmonary arterial hypertension, Pulmonary Hypertension News, July 12, 2025

Researchers have found two specific genes — PRDX1 and TNFAIP3 — whose activity levels could serve as diagnostic biomarkers that could help doctors detect idiopathic pulmonary arterial hypertension (IPAH) earlier and possibly guide treatment. This study, published in Scientific Reports on July 12, 2025, focused on genes linked to ferroptosis, an iron-dependent type of cell

New study finds possible diagnostic biomarkers for idiopathic pulmonary arterial hypertension, Pulmonary Hypertension News, July 12, 2025 Read Post »

Gaps In Access to pulmonary hypertension care and Opportunities for improvement: A multi-site qualitative study, BMC Pulmonary Medicine, July 28, 2025

A multi-site qualitative study exploring both patient and provider perspectives on access to and timeliness of pulmonary hypertension (PH) care across the full care continuum—from symptom onset through long-term management was recently published in BMC Pulmonary Medicine. Interviews with 41 participants (21 patients, 20 providers) at three U.S. expert pulmonary hypertension centers revealed persistent delays

Gaps In Access to pulmonary hypertension care and Opportunities for improvement: A multi-site qualitative study, BMC Pulmonary Medicine, July 28, 2025 Read Post »

Programmed Cell Death: A Pivotal Player in Pulmonary Arterial Hypertension, European Journal of Pharmacology, July 8, 2025

In pulmonary arterial hypertension (PAH) lung blood vessel cells grow excessively, increasing vascular resistance and ultimately leading to right heart failure. While researchers understand many molecular mechanisms behind pulmonary arterial hypertension, effective treatments remain limited. Programmed cell death (PCD) plays a crucial role in the pathogenesis of various diseases, including cancer, chronic conditions, cardiovascular disorders,

Programmed Cell Death: A Pivotal Player in Pulmonary Arterial Hypertension, European Journal of Pharmacology, July 8, 2025 Read Post »

First robotic lung transplant in Abu Dhabi, UAE, Gulf Today, July 8, 2025

Cleveland Clinic Abu Dhabi has achieved a historic milestone by performing the Gulf region’s first robotic lung transplants, making it one of only five centers worldwide to successfully complete this advanced procedure. Two patients with idiopathic pulmonary fibrosis and secondary pulmonary hypertension have undergone the surgery. These surgeries were quite complex due to pulmonary hypertension,

First robotic lung transplant in Abu Dhabi, UAE, Gulf Today, July 8, 2025 Read Post »

The Pulmonary Hypertension Global Patient Survey: understanding the experiences and perspectives of patients, European Respiratory Journal Open Research 2025

The key findings from the Pulmonary Hypertension Global Patient Survey (PHGPS) adult cohort of 3,329 patients have been published in the ERJ Open Research 2025, highlighting the achievements, shortcomings & regional disparities of clinical care & research globally. The Pulmonary Hypertension Global Patient Survey represents the most extensive survey of its type in the pulmonary

The Pulmonary Hypertension Global Patient Survey: understanding the experiences and perspectives of patients, European Respiratory Journal Open Research 2025 Read Post »

Phase 3 pulmonary arterial hypertension trial of ralinepag now fully enrolled, results are expected in 2026, Pulmonary Hypertension News, June 27, 2025

Ralinepag is a novel prostacyclin receptor agonist. It is designed to mimic prostacyclin, a molecule that reduces blood pressure by relaxing blood vessels. It is a once-daily oral formulation. United Therapeutics has announced the completion of enrollment for its ADVANCE OUTCOMES, a Phase 3 clinical trial testing ralinepag for pulmonary arterial hypertension (PAH). The study

Phase 3 pulmonary arterial hypertension trial of ralinepag now fully enrolled, results are expected in 2026, Pulmonary Hypertension News, June 27, 2025 Read Post »

New study investigates reliability of point of care and remote monitoring for pulmonary arterial hypertension biomarkers NT-proBNP/BNP , Respirology, July 3, 2025

Brain natriuretic peptide (BNP) and N-terminal prohormone of BNP (NT-proBNP) serve as crucial biomarkers in pulmonary arterial hypertension (PAH). Nevertheless, test results are seldom accessible during clinical evaluations. A study published on Respirology on July 3, 2025, examined the reliability of NT-proBNP/BNP point-of-care tests (POCT) in pulmonary arterial hypertension patients and assessed the stability of

New study investigates reliability of point of care and remote monitoring for pulmonary arterial hypertension biomarkers NT-proBNP/BNP , Respirology, July 3, 2025 Read Post »

The landscape of referrals for lung transplantation in pulmonary arterial hypertension: a report from the Pulmonary Hypertension Association Registry, Journal of Heart and Lung Transplantation, June 26, 2025

A large US registry study of 1,671 patients with pulmonary arterial hypertension (PAH) over 4607 person-years of follow up found that lung transplant referrals are occurring far too infrequently and too late in the disease course. Despite professional society recommendations for early referral, only 12% of patients were referred for transplantation over the study period.

The landscape of referrals for lung transplantation in pulmonary arterial hypertension: a report from the Pulmonary Hypertension Association Registry, Journal of Heart and Lung Transplantation, June 26, 2025 Read Post »

Scientists create first mini-lungs with built-in blood vessels, unlocking new insights for pulmonary vascular disease, UCLA Newsroom, July 2, 2025

Researchers at the University of California, Los Angeles (UCLA), have achieved a breakthrough by successfully growing miniature lungs from stem cells that include functioning blood vessel networks for the first time. Published in Cell, this advancement represents a significant step forward because previous lung organoids lacked the essential vascular systems needed for proper lung function.

Scientists create first mini-lungs with built-in blood vessels, unlocking new insights for pulmonary vascular disease, UCLA Newsroom, July 2, 2025 Read Post »

Computer modeling used in study to simulate long-term mortality/morbidity in patients with pulmonary arterial hypertension If sotatercept Is delayed, Advances in Therapy, June 17, 2025

Researchers used computer modeling to compare immediate versus delayed (2-year) treatment with sotatercept, a new pulmonary arterial hypertension medication, to predict lifetime outcomes. Study Method: Building on the findings of the phase 3 STELLAR trial on sotatercept the authors of the study refer to a population health model which was recently published assessing the long-term

Computer modeling used in study to simulate long-term mortality/morbidity in patients with pulmonary arterial hypertension If sotatercept Is delayed, Advances in Therapy, June 17, 2025 Read Post »

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