Inhaled treprostinil for treating pulmonary hypertension with interstitial lung disease including combined pulmonary fibrosis and emphysema Inhaled treprostinil for treating pulmonary hypertension with interstitial lung disease including combined pulmonary fibrosis and emphysema - NIHR Innovation Observatory * Who we are * What we do * Our Networks * Engage * Events * News * Resources Get January 2024 Inhaled treprostinil for treating pulmonary hypertension with interstitial lung disease including combined pulmonary fibrosis and emphysemaPulmonary hypertension is a rare and serious condition that occurs when blood pressure within the lungs becomes abnormally high. It can be caused by thickening of the pulmonary artery walls, heart failure, lung disease and clots within the lungs' blood
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Systematic evaluation of subgroup analyses of inhaled treprostinil in pulmonary hypertension due to interstitial lung disease. The INCREASE trial introduced a novel therapeutic option for Pulmonary Hypertension caused by Interstitial Lung Disease. Subsequently to this trial, several subgroup analyses were conducted, aiming to explore specific effects within subgroups. This study aimed
Oral Treprostinil is Associated with Improved Survival in FREEDOM-EV and its Open-Label Extension. In the event-driven FREEDOM-EV trial, oral treprostinil delayed clinical worsening in patients with pulmonary arterial hypertension (PAH). Open-label extension studies offer additional data about tolerability, efficacy, and survival, especially for those initially assigned placebo. The aim of the current study was to determine if oral treprostinil changed survival when considering the parent and extension study, if treprostinil provides functional benefits for participants initially assigned to placebo, and if the benefits observed for those treated with treprostinil were durable. Both active and placebo participants from FREEDOM-EV could enroll in the FREEDOM-EV open-label extension (OLE) study
Short term effect of intravenous treprostinil in term and preterm infants with pulmonary hypertension. Pulmonary hypertension (PH) is a life-threatening condition in newborns. We aimed to assess the clinical and echocardiographic responses of term and preterm infants to treprostinil. This retrospective study included newborns diagnosed with PH and treated with treprostinil as additional therapy after inhaled nitric oxide administration in the neonatal intensive care unit of a tertiary center. Term and preterm infants were compared in terms of echocardiographic findings and clinical findings 4 weeks after treprostinil treatment. During the study period, 11 term and 18 preterm infants were diagnosed with PH and received treprostinil. There were no differences in the echocardiographic findings
Inhaled treprostinil in patients with pulmonary hypertension associated with interstitial lung disease with less severe haemodynamics: a post hoc analysis of the INCREASE study. Inhaled treprostinil (iTre) is the only treatment approved for pulmonary hypertension due to interstitial lung disease (PH-ILD) to improve exercise capacity. This post hoc analysis evaluated clinical worsening and PH-ILD
Inhaled Treprostinil in Pulmonary Hypertension Associated with COPD: PERFECT study results. Pulmonary hypertension accompanying chronic obstructive pulmonary disease (PH-COPD) is associated with worse outcomes than COPD alone. There are currently no approved therapies to treat PH-COPD. The PERFECT study (NCT03496623) evaluated the safety and efficacy of inhaled treprostinil (iTRE
Treatment with Oral or Inhaled Treprostinil in Patients with Pulmonary Arterial Hypertension and Cardiovascular Comorbidities. An increasing number of patients with pulmonary arterial hypertension (PAH) have cardiovascular comorbidities. However, the effects of comorbidities on responses to PAH treatment are not well understood. Do cardiovascular comorbidities in patients with PAH influence the efficacy and tolerability of inhaled or oral treprostinil? All patients from phase 3 studies TRIUMPH (N = 235) and FREEDOM-EV (N = 690) were included in this post hoc analysis and were classified as having 0, ≥1, or ≥2 cardiovascular comorbidities of interest based on patients' medical histories. The mean difference in 6-minute walk distance (6MWD) and N-terminal pro-brain natriuretic peptide (NT-proBNP
Plain language summary of the INCREASE study: inhaled treprostinil (Tyvaso) for the treatment of pulmonary hypertension due to interstitial lung disease. Here, we summarize the results from the INCREASE study, originally published in the . The INCREASE study looked at how well a medication called inhaled treprostinil works and how safe it is, compared to placebo (a fake medication), in adults who have pulmonary hypertension associated with interstitial lung disease or PH-ILD. A total of 326 participants took part in the study. Half the participants took inhaled treprostinil and the other half took placebo. After 16 weeks, the INCREASE study showed that participants with PH-ILD who took inhaled treprostinil could walk around 31 meters (102 feet) further than the participants who took
Treprostinil in neonates with congenital diaphragmatic hernia-related pulmonary hypertension. To describe our experience with treprostinil, evaluate correlations with cardiac function, and assess for adverse effects in neonates with congenital diaphragmatic hernia-related pulmonary hypertension (CDH-PH). A retrospective review of a single-center prospective registry at a quaternary care children's hospital. Patients included in the study had CDH-PH treated with treprostinil between April 2013 and September 2021. Assessed outcomes were brain-type natriuretic peptide (BNP) levels and quantitative echocardiographic parameters collected at baseline, one week, two weeks, and one month after treprostinil initiation. Right ventricular (RV) function was assessed by tricuspid annular plane
Safety and efficacy of transitioning from selexipag to oral treprostinil in pulmonary arterial hypertension: Findings from the ADAPT registry. Oral treprostinil and selexipag are drugs targeting the prostacyclin pathway and are approved for treatment of pulmonary arterial hypertension (PAH). In the setting of unsatisfactory clinical response or side effects while on selexipag, there is little data on clinical benefit, safety, or strategies on transitioning to oral treprostinil. Using prospective data from the ADAPT registry, we aimed to evaluate clinical outcomes, safety, and transition strategies in ten patients with PAH transitioning from selexipag to oral treprostinil. ADAPT was a prospective, real-world, multicenter, United States-based registry of patients with PAH newly started
Transition from Intravenous Epoprostenol to Treprostinil Due to Intolerable Side Effects in Patients With Pulmonary Arterial Hypertension. Intravenous epoprostenol improves exercise capacity and survival in patients with pulmonary arterial hypertension (PAH); however, it has side effects. Reviewing the side effects associated with epoprostenol and treprostinil is essential for improving the long -term treatment strategies for PAH. This retrospective review included patients with PAH who transitioned from intravenous epoprostenol to intravenous treprostinil owing to intolerable side effects, including high cardiac output symptoms, ascites, and thrombocytopenia. Of the 85 patients who received epoprostenol at our hospital between 2013 and 2021, 16 (11 women), with a median age of 33 (range 26
The risk profile change in patients with severe chronic thromboembolic pulmonary hypertension treated with subcutaneous treprostinil. Chronic thromboembolic pulmonary hypertension (CTEPH) is successfully treatable with pulmonary endarterectomy (PEA), balloon pulmonary angioplasty, and medical therapy. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management risk score (RRS) is able to predict long-term outcome in inoperable patients or in patients with residual PH after surgery. We performed a post hoc analysis of RRS in patients who were enrolled in the CTREPH study (NCT01416636), a randomized, double-blind clinical trial comparing high-dose and low-dose subcutaneous (SC) treprostinil in patients with severe CTEPH that was classified
Long-term inhaled treprostinil for PH-ILD: INCREASE open-label extension study. The 16-week randomised, placebo-controlled INCREASE trial (RCT) met its primary endpoint by improving 6-minute walk distance (6MWD) in patients receiving inhaled treprostinil for pulmonary hypertension due to interstitial lung disease (PH-ILD). The open-label extension (OLE) evaluated long-term effects of inhaled treprostinil in PH-ILD. Of 258 eligible patients, 242 enrolled in the OLE and received inhaled treprostinil. Assessments included 6MWD, pulmonary function testing, N-terminal pro-brain natriuretic peptide (NT-proBNP), quality of life, and adverse events. Hospitalizations, exacerbations of underlying lung disease, and death were recorded. At OLE baseline, patients had a median age of 70 years and a mean 6MWD
Inhaled Treprostinil in Pulmonary Hypertension Due to Interstitial Lung Disease. No therapies are currently approved for the treatment of pulmonary hypertension in patients with interstitial lung disease. The safety and efficacy of inhaled treprostinil for patients with this condition are unclear. We enrolled patients with interstitial lung disease and pulmonary hypertension (documented by right heart catheterization) in a multicenter, randomized, double-blind, placebo-controlled, 16-week trial. Patients were assigned in a 1:1 ratio to receive inhaled treprostinil, administered by means of an ultrasonic, pulsed-delivery nebulizer in up to 12 breaths (total, 72 μg) four times daily, or placebo. The primary efficacy end point was the difference between the two groups in the change in peak 6
Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension Continuous subcutaneous treprostinil for treatment of pulmonary arterial hypertension ..
Prenatal treprostinil reduces the pulmonary hypertension phenotype in the rat model of congenital diaphragmatic hernia. Persistent pulmonary hypertension (PH) causes significant mortality and morbidity in infants with congenital diaphragmatic hernia (CDH). Since pulmonary vascular abnormalities in CDH develop early during foetal development, we hypothesized that prenatal maternal administration of treprostinil, through its anti-remodelling effect, would improve the PH-phenotype in the nitrofen rat model of CDH. In a dose-finding study in normal, healthy pregnant rats, we demonstrated target-range foetal plasma treprostinil concentrations without signs of toxicity. Next, an efficacy study was performed assessing the effects of treprostinil administration at 900 and 1500ng/kg/min from gestational day
Oral treprostinil improves pulmonary vascular compliance in pulmonary arterial hypertension. Oral treprostinil has been shown to improve exercise capacity and delay disease progression in patients with pulmonary arterial hypertension (PAH), but its effects on hemodynamics are not well-characterized. The FREEDOM-EV trial was a Phase III, international, placebo-controlled, double-blind, event -driven study in 690 participants with PAH who were taking a single oral PAH therapy. FREEDOM-EV demonstrated a significantly reduced risk for clinical worsening with oral treprostinil taken three times daily and did not uncover new safety signals in PAH patients. Sixty-one participants in the FREEDOM-EV trial volunteered for a hemodynamics sub-study. Pulmonary artery compliance (PAC), a ratio of stroke
Study design and rationale for the TETON phase 3, randomised, controlled clinical trials of inhaled treprostinil in the treatment of idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) greatly impacts quality of life and eventually leads to premature death from respiratory failure. Inhaled treprostinil was associated with improvements in forced vital capacity (FVC) and reduced exacerbations of underlying lung disease in post hoc analyses from a phase 3 study in patients with precapillary pulmonary hypertension due to interstitial lung disease. These results, combined with preclinical evidence of treprostinil's antifibrotic activity, support its investigation in the treatment of IPF. The TETON programme consists of two replicate, 52-week, randomised, double-blind placebo-controlled