Yutrepia (treprostinil inhaled dry powder) / Liquidia Technologies - LARVOL DELTA

Safety of Low-Dose Oral Treprostinil in PH-HFpEF Supports Use in Patients with PAH with Cardiovascular Comorbidities (ISHLT 2025) - P3 | "Recent analyses of the FREEDOM-EV and TRIUMPH trials demonstrated no difference in treatment response or tolerability of oral and inhaled treprostinil (TRE) with increasing CV comorbidity burden...No oral TRE patients died during the study; one (2%) PBO patient died during the study.Conclusion In SOUTHPAW, AEs were consistent with prior TRE studies, and there were no concerning safety signals with low doses of oral TRE (study maximum dose 4 mg TID). This suggests that oral TRE may be safely used in PAH patients with CV comorbidities and risk factors for HFpEF."

Clinical • Cardiovascular • Congestive Heart Failure • Fatigue • Heart Failure • Pain • Pulmonary Disease

Treprostinil: Enhancing Pre-Transplant Optimization for Lung Transplant Candidates (ISHLT 2025) - "Our first patient saw a 153.8% increase in her 6MWT, and our second patient saw a 99.84% increase. These cases support using inhaled treprostinil to improve patients' exercise capacity, which has important implications both pre- and post-transplant."

Pre-transplantation • Cardiovascular • Fibrosis • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sarcoidosis • Scleroderma • Systemic Sclerosis • Transplantation

CONCURRENT PULMONARY FIBROSIS AND PULMONARY ARTERIAL HYPERTENSION APPROACHED BY CARDIOPULMONARY EXERCISE TESTING, AN ILLUSTRATIVE CASE REPORT - Abdelrahman Abou-Shakra (ACC 2025) - "PAH can coexist in patients with IPF. CPET emerges as a test to clearly distinguish between PAH and IPF as the etiology of dyspnea."

Case report • Clinical • Cardiovascular • Hypertension • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

Liquidia Corporation Announces FDA Acceptance of New Drug Application Resubmission for YUTREPIA (treprostinil) Inhalation Powder (GlobeNewswire) - "Liquidia Corporation...today announced that the U.S. Food and Drug Administration (FDA) has accepted its New Drug Application (NDA) resubmission for YUTREPIA (treprostinil) inhalation powder to treat pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease (PH-ILD). The FDA confirmed that the resubmission was a complete, Class 1 response to the previous action letter issued on August 16, 2024, which granted tentative approval of YUTREPIA for both PAH and PH-ILD. The FDA has set a Prescription Drug User Fee Act (PDUFA) goal date of May 24, 2025."

FDA filing • PDUFA • Pulmonary Arterial Hypertension

Liquidia Announces Poster Presentations at the American Thoracic Society (ATS) 2025 International Conference (GlobeNewswire) - "Liquidia Corporation...today announced the company will present three posters at the American Thoracic Society (ATS) 2025 International Conference, taking place May 18-21, 2025, in San Francisco. Two posters will highlight new data from the company’s open-label ASCENT study of LIQ861 (YUTREPIA) in PH-ILD patients with a focus on safety, tolerability, exploratory changes in 6-minute walk distance, quality of life, and changes in cardiac effort. A third poster is a case study of a patient with PAH participating in the open-label extension study (INSPIRE)."

Clinical data • Pulmonary Arterial Hypertension

An Ascent to Week 8: Initial Safety and Exploratory Efficacy Data on LIQ861 Dry Powder Inhaled Treprostinil in Ph-ILD Patients (ATS 2025) - No abstract available

Clinical • Interstitial Lung Disease

Transitioning From Parenteral Treprostinil to LIQ861 in a Patient With PAH (ATS 2025) - No abstract available

Clinical • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Teton Phase 3 Clinical Trials of Inhaled Treprostinil in the Treatment of Idiopathic Pulmonary Fibrosis: Annual Update of Preliminary Baseline Data (ATS 2025) - No abstract available

Clinical • P3 data • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases

TETON-PPF Clinical Trial of Inhaled Treprostinil for the Treatment of Progressive Pulmonary Fibrosis: Preliminary Baseline Demographics (ATS 2025) - No abstract available

Clinical • Immunology • Pulmonary Disease • Respiratory Diseases

PULMONARY HYPERTENSION, RIGHT-TO-LEFT ATRIAL SHUNTING, AND THE COMPLEX INTERPLAY OF OXYGENATION (SCCM 2025) - "A multidrug regimen of Sildenafil, Treprostinil, Inhaled Nitric Oxide, and loop diuretics was initiated. Despite this, the patient progressed to right ventricular (RV) failure requiring Dobutamine...Increasing SVR with vasopressors can improve hypoxemia but may worsen heart failure, as seen in this patient, potentially leading to death. This case underscores the complexity of managing PH with RL shunting, emphasizing the need for personalized treatment and further research to understand the pathophysiology and develop targeted interventions for this intricate clinical scenario."

Cardiovascular • Congestive Heart Failure • Heart Failure • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Extension Study for Participants in LIQ861 Trials to Evaluate the Long-term Safety of Dry Powder Inhalation of Treprostinil (clinicaltrials.gov) - P3 | N=92 | Active, not recruiting | Sponsor: Liquidia Technologies, Inc. | Trial completion date: Mar 2022 ➔ May 2025 | Trial primary completion date: Dec 2021 ➔ May 2025

Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Inhaled Treprostinil for ILD-PH. (CVCT USA 2024) - No abstract available

Interstitial Lung Disease

Chronic Obstructive Pulmonary Hypertension Inhaled Treprostinil for COPD-PH: PERFECT trial. (CVCT USA 2024) - No abstract available

Cardiovascular • Chronic Obstructive Pulmonary Disease • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Efficacy of pulmonary vasodilators for the prevention of acute exacerbations on interstitial pneumonia with pulmonary hypertension (APSR 2024) - "While efficacy of inhaled treprostinil in reducing FVC decline in PH-IP patients has been reported, the impact of pulmonary vasodilator therapy on acute exacerbations of IP (AE-IP) remains unclear. The use of pulmonary vasodilators in patients with PH-IP may have the potential to prevent AE-IP, suggesting a new therapeutic strategy for managing these challenging conditions."

Clinical • Cardiovascular • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

Conceptualization and Validation of a De Novo Cost-Effectiveness Analysis for Pulmonary Hypertension Associated With Interstitial Lung Disease (ISPOR-EU 2024) - P2/3 | "The 16-week INCREASE randomised control trial (NCT02630316) and open-label extension (NCT02633293) evaluated the safety and efficacy of inhaled treprostinil in PH-ILD. A cohort PSM using patient-level data from INCREASE to inform survival curves predicting the time to disease progression events and death was considered most appropriate."

Cost effectiveness • HEOR • Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

INHALED TREPROSTINIL FOR THE TREATMENT OF CONNECTIVE TISSUE-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION (CHEST 2024) - P3 | "Eligible patients were 18 to 75 years old with idiopathic or familial PAH or PAH associated with CTD, HIV, or anorexigen use receiving background bosentan or sildenafil at a stable dose for at least 3 months. Following 12 weeks of treatment with inhaled treprostinil in the TRIUMPH study, patients with CTD-PAH had comparable improvements in 6MWD and reductions in NT-proBNP relative to those without CTD-PAH. No patients experienced worsening in FC and safety was similar between the groups. CLINICAL IMPLICATIONS: These data support the beneficial use of inhaled treprostinil in patients with CTD-PAH."

Cardiovascular • Human Immunodeficiency Virus • Infectious Disease • Pulmonary Arterial Hypertension • Respiratory Diseases • Rheumatology

7250: TYVASO DPI® (treprostinil inhalation powder): Clinical Pearls and Drug-Device Characteristics (CHEST 2024) - "Description: This event brings together renowned experts and experienced clinicians at the forefront of PAH and PH-ILD care and research. We aim to review clinical evidence related to inhaled prostacyclin therapy and discuss patient selection, side effect management, transitions between different formulations, and drug-device technical characteristics."

Clinical

SILDENAFIL-INDUCED ACUTE PSYCHOSIS IN A PATIENT WITH PULMONARY HYPERTENSION: A RARE COMPLICATION (CHEST 2024) - "He was prescribed inhaled Treprostinil four times daily and oral sildenafil 20 mg three times daily.In the past three weeks, patient had progressive symptoms of mania, pressured speech, paranoia, insomnia, flight of ideas, and akathisia, concerning for true psychosis...Empiric treatment with quetiapine 25 mg every 8 hours and as needed haloperidol 5 mg was used to control acute psychotic episodes... Sildenafil is a common medication prescribed for pre-capillary pulmonary hypertension. When considering sildenafil, sudden onset neuropsychiatric side effects should be discussed with patients and families. Although extremely rare, psychotic symptoms tend to manifest shortly after initiation, workup is usually unremarkable, and patients gradually return to baseline with cessation of Sildenafil."

Clinical • Anesthesia • Bipolar Disorder • Cardiovascular • CNS Disorders • Congestive Heart Failure • Heart Failure • Immunology • Insomnia • Interstitial Lung Disease • Mental Retardation • Mood Disorders • Pain • Psychiatry • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sleep Disorder

LONG-TERM OUTCOMES AND DOSING IN THE BREEZE STUDY OPTIONAL EXTENSION PHASE (CHEST 2024) - P1 | "In patients with PAH, Tyvaso DPI was safe and well-tolerated. The persistent benefit on 6MWD may be attributed to the up-titrated dose over time, which has been seen in other studies of inhaled treprostinil, or alternatively may be associated with improved compliance. CLINICAL IMPLICATIONS: Tyvaso DPI is a safe and tolerable treatment option for patients with PAH that may provide long-term maintenance of clinical stability."

Cough • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

CHARACTERISTICS AND CLINICAL IMPLICATIONS OF ANTI-TH/TO ANTIBODY IN SYSTEMIC SCLEROSIS: A SINGLE-CENTER ILD COHORT STUDY (CHEST 2024) - "WHO Group 1 pulmonary arterial hypertension was present in only 11% of patients, confirmed by right heart catheterization and treated with inhaled Treprostinil and Tadalafil...55% of patients were on Mycophenolate mofetil and 44% were on Nintedanib along with other immunosuppression... Our series reveals that patients with Anti-Th/To antibody tend to have female predominance, present with Raynaud's phenomenon, esophageal dysfunction and develop pulmonary involvement. Female predominance noted in our cohort contrasts the findings of Ceribelli et al who noted a male predominance, but aligns with Suresh et al. While only 11% of patients developed pulmonary arterial hypertension, Suresh et al showed over one-third of patients developing pulmonary hypertension on long term follow up."

Clinical • Bronchiectasis • Cardiovascular • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension • Rheumatology • Scleroderma • Systemic Sclerosis

RAPID INPATIENT UPTITRATION OF INHALED TREPROSTINIL IN PATIENTS WITH SEVERE PH-ILD (CHEST 2024) - "Our study introduces a novel approach in the management of severe PH-ILD through rapid inpatient uptitration of inhaled treprostinil. Achieving therapeutic doses within a week, as opposed to the conventional 8 to 16 weeks, not only demonstrates feasibility and safety but also indicates a potential for significant patient outcome improvements. CLINICAL IMPLICATIONS: This method could redefine treatment protocols for severe PH-ILD and warrants further investigation through larger, longitudinal studies to confirm its efficacy and safety."

Clinical • Cardiovascular • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension

BASELINE CHARACTERISTICS OF PATIENTS ENROLLED IN THE ASCENT STUDY: EVALUATING SAFETY AND TOLERABILITY OF YUTREPIA, A DRY-POWDER INHALED TREPROSTINIL IN PH-ILD (CHEST 2024) - P | "The present study reports the baseline demographics and clinical characteristics of the first seven patients enrolled in the ASCENT study. This study will enroll approximately 60 subjects with prostacyclin naïve WHO group 3 PH-ILD to be treated with LIQ861for up to 52 weeks. Additional exploratory endpoints will be collected including patient reported outcomes (EmPHasis10, Dyspnea 12, and simplified cough score), WHO FC, NT-proBNP, 6MWD, cardiac effort, quantitative CT Chest scan with functional respiratory imaging, PFTs, peak inspiratory flow rate, and echocardiogram parameters."

Clinical • Cardiovascular • CNS Disorders • Cough • Insomnia • Interstitial Lung Disease • Pain • Pulmonary Arterial Hypertension • Sleep Disorder

DON'T FORGET THE KNEES: NOVEL TBX4 GENE MUTATION ASSOCIATED WITH PULMONARY ARTERIAL HYPERTENSION (CHEST 2024) - "The patient was started on triple combination therapy that included macitentan, riociguat (titrated to 2.5 mg TID) and inhaled treprostinil dry powdered inhaler (titrated to 64mcg QID). TBX4 gene mutation should be considered in PAH patients especially when associated with skeletal abnormalities. Careful history taking and clinical examination can provide important clues."

Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases • Rheumatology

USING DOBUTAMINE AS A BRIDGE TO INHALED TREPROSTINIL IN SEVERE PULMONARY HYPERTENSION DUE TO INTERSTITIAL LUNG DISEASE (CHEST 2024) - "In severe PH-ILD patients, inotropic support with Dobutamine allowed for the target dose of inhaled treprostinil to be achieved, ultimately resulting in improvements in PVR, CI, and TAPSE at 3-month follow-up. CLINICAL IMPLICATIONS: These findings suggest that in severe PH-ILD with RV dysfunction, adjunctive use of Dobutamine during the initiation and up-titration of inhaled treprostinil is a viable therapeutic strategy to augment RV inotropy until the pulmonary vasodilator has taken effect and worked to decrease PVR. However, large-scale studies are warranted to confirm these preliminary results and to explore further the potential benefits, optimal dosing, and safety profile of this treatment approach in this patient population."

Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

EXPLORING THE INCREASED SERIOUS ADVERSE EVENTS ASSOCIATED WITH INHALED TREPROSTINIL: AN FDA ADVERSE EVENT REPORTING SYSTEM DATABASE ANALYSIS (CHEST 2024) - "Our study showed similar results in terms of mild to moderate AEs including cough, dyspnea, headche, diziness. However unlike the INCREASE trial showing 23.3% serious AEs our study showed 60% serious AEs. The underlying mechanism of increased serious AEs are unknown however our results show increase association with heart failure, chest pain and hypotesion."

Adverse events • Serious adverse event • Cardiovascular • CNS Disorders • Congestive Heart Failure • Cough • Heart Failure • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases