Clinical Trials

The purpose of this study is to assess the safety and performance of the Aria CV Pulmonary Hypertension System in patients with pulmonary arterial hypertension.

The purpose of this study is to evaluate the safety and efficacy of AV-101 (dry powder inhaled imatinib) in patients with Pulmonary Arterial Hypertension (PAH). The Phase 2b part of the study will assess three doses to establish an optimal dose for the Phase 3 part of the study. The Phase 2b primary endpoint will be the placebo corrected change in pulmonary vascular resistance (PVR). The Phase 3 primary endpoint will be the placebo corrected change in 6-minute walk distance (6MWD) after 24 weeks of treatment.

The primary objective of this study is to evaluate the long-term safety of LIQ861 in patients with pulmonary arterial hypertension (PAH).

Study ROR-PH-303, ADVANCE EXTENSION, is an open-label extension (OLE) study for participants with WHO Group 1 PAH who have participated in another Phase 2 or Phase 3 study of ralinepag.

The purpose of this study is to see if patients with Pulmonary Arterial Hypertension related to Interstitial Lung Disease (ILD-PAH) will have impaired cardiac output reserve and leg blood flow during exercise, and inhlaed treprostinil will acutely increase exertional leg blood flow and cardiac output with preserved skeletal muscle O2 uptake efficiency, and to see a baseline hemodynamic improvement in exertional cardiac output with inhaled treprostinil will predict subsequent NYHA class improvement at 3 months with chronic inhaled treprostinil therapy.

The purpose of this study is to evaluate the prevalence and effect of frailty in patients referred to pulmonary hypertension (PH) clinic at our institution.

The purpose of this study is to evaluate the effects of RT234 on exercise parameters assessed by a specialized exercise test (Cardiopulmonary Exercise Test or CPET) in patients with pulmonary arterial hypertension (PAH).

The purpose of this study is to assess the effect of Treprostinil Palmitil Inhalation Powder (TPIP) compared with placebo on pulmonary vascular resistance (PVR).

The purpose of this study is to extend innovation to the right heart and develop a '4D RightHeart virtual catheter (4D RH vCath).

The purpose of this study is to assess known gene variants in a patient with associated pulmonary arterial hypertension (PAH) due to scleroderma who is clinically vasoreactive to compare to published variants that are predictive of acute vasoreactivity in idiopathic PAH.
 

The purpose of this study is to determine if non-invasive measurement of blood flow to the lungs and the movement of gas from the lungs to the blood stream at rest and in response to low to moderate intensity exercise can be used as an assessment tool to allow for earlier detection and monitoring of individuals with pulmonary-arterial hypertension, and the response to drug treatments.

The purpose of this study is to:

1. To assess out of pocket expenditures of patients on medical therapy for Group 1 PAH, its impact on medication affordability and whether that adversely affected treatment regimens.

2. To analyze potential effects associated with the financial burden of PAH medical therapy.

3. The secondary outcome is to assess the impact of pulmonary arterial hypertension on healthy lifestyle of the patients.

STUDY OBJECTIVES Primary objective(s) To evaluate the effect of macitentan 10 mg on pulmonary vascular resistance (PVR) as compared to placebo in subjects with pulmonary hypertension (PH) after left ventricular assist device (LVAD) implantation.

Secondary objectives To evaluate the effect of macitentan 10 mg as compared to placebo on cardio-pulmonary hemodynamics and disease severity in subjects with PH after LVAD implantation.

To evaluate the safety and tolerability of macitentan 10 mg in subjects with PH after LVAD implantation.

Exploratory objectives To explore the potential effect of macitentan 10 mg as compared to placebo on right ventricular function ...

This study is being conducted to assess the long-term safety, tolerability, and efficacy of sotatercept (MK-7962, formerly called ACE-011) in participants with Pulmonary Arterial Hypertension (PAH). This open-label, long-term follow-up (LTFU) study is supported by data from the PULSAR study (Phase 2, NCT03496207) in which treatment with sotatercept resulted in hemodynamic and functional improvements in the study participants, including those receiving maximal PAH therapy with double/triple drug combinations and intravenous prostacyclin.

The primary objective of this open-label, LTFU study is to evaluate the long-term safety and tolerability of sotatercept when added to background PAH therapy in adult participants with PAH who ...

The purpose of this study is to compare the effectiveness and safety of three (macitentan, tadalafil, and selexipag) versus two (macitentan and tadalafil) oral treatments for patients who have newly diagnosed and untreated hypertension of the pulmonary artery.

The purpose of this extension study is to provide data on the long-term safety, tolerability, and efficacy of imatinib in the treatment of severe pulmonary arterial hypertension.

Pulmonary hypertension (PH) is a progressive, symptomatic, and ultimately fatal disorder for which there are no effective therapies. Resident gut microbiota are now recognized as potent modifiers of the host immune responses in various pathologies. Evidence from animal studies suggest that the microbial composition is altered in lung diseases such as asthma, chronic obstructive pulmonary disorders (COPD) and cystic fibrosis. Our goal in this study will be to determine and compare the gut microbial composition between healthy individuals and pulmonary hypertensive patients.

The purpose of this study is to establish a collection, termed biorepository or biobank, of samples as well as both clinical and genetic data from patients with pulmonary arterial hypertension.

The purpose of this study is to evaluate the diagnostic benefit of adding exercise to cardiac Magnetic Resonance Imaging to help show early changes that may assist in the earlier diagnosis of Pulmonary Artery Hypertension.

The purpose of this study is to evaluate the Pulmonary Hypertension Functional Class Self Report (PHFC-SR) ratings for a sample of subjects with pulmonary hypertension (PH).

A secondary purpose is to explore the concordance between the patient-reported PH-FC-SR and clinician-assessed World Health Organization Functional Classification (WHO-FC) measure in a sample of subjects with PH, and to assess the construct validity between the patient-reported PH-FC-SR and a patient-reported disease-specific measure and a general health-related quality of life (HRQOL) measure.

The purpose of this study is to characterize the clinical and hemodynamic response of PAH therapy in patients with atypical PAH and risk factors for left heart disease.

The purpose of this study is to evaluate the safety, pharmacokinetics (PK), pharmacodynamics (PD), and biomarkers of inhaled GB002 in adults with World Health Organization (WHO) Group 1 Pulmonary Arterial Hypertension (PAH).

The objectives of this study are to measure and improve quality of care (including assessing differences in adherence to evidence-based guidelines and establishing benchmarks for health outcomes); determine the clinical effectiveness, comparative effectiveness, and cost effectiveness of treatment approaches; understand risk factors for outcomes and regional/center differences; and facilitate funded clinical trials of new therapies and collaboration with the PAH community at large, including providers, patients, and their caregivers.

The goals of the PHAR include 1) measuring and improving quality of care (including assessing differences in adherence to evidence-based guidelines and establishing benchmarks for health outcomes), 2) determining the clinical effectiveness, comparative effectiveness, and cost effectiveness of treatment approaches, 3) understanding risk factors for outcomes and regional/center differences, and 4) facilitating funded clinical trials of new therapies and collaboration with the PAH community at large, including providers, patients, and their caregivers.

The purpose of this extension study is to evaluate the long-term effects of GB002 (seralutinib) in subjects who previously participated in a GB002 PAH study.

The purpose of this study is to evaluate the effect of Rodatristat Ethyl from baseline on pulmonary vascular resistance as measured at right heart catheterization. 

The purpose of this study is to identify the molecular basis of various forms of heart and lung disease, particularly those that have varying degrees of pulmonary hypertension, pulmonary vascular remodeling, and right ventricular dysfunction.

The purpose of this study is to demonstrate superiority of macitentan 75 milligrams (mg) in prolonging the time to the first clinical events committee (CEC)-adjudicated morbidity or mortality (M/M) event in participants with symptomatic pulmonary arterial hypertension (PAH) compared to macitentan 10 mg.

We propose to develop a biorepository of plasma samples from patients with suspected and confirmed portopulmonary hypertension to improve our understanding of this disease and its response to pulmonary arterial hypertension targeted therapy and liver transplantation.

The purpose of this study is to evaluate the effect of sotatercept (ACE-011) in adults with Pulmonary Arterial Hypertension. Each eligible participant will receive standard of care (SOC) plus sotatercept (ACE-011) for a 24 week treatment period followed by a 16 week follow up period.

The primary purpose of this study is to evaluate the long-term safety and tolerability of LIQ861, a dry powder formulation of treprostinil, in patients with Pulmonary Arterial Hypertension (PAH). A secondary objective of this study is to evaluate the comparative bioavailability of treprostinil between two formulations of inhaled therapy.

The purpose of this study is to evaluate pulmonary arterial hypertension (PAH) subjects on a stable regimen of Tyvaso after switching to a corresponding dose of TreT.

The purpose of this study is to identify, characterize, and study a group of patients who have pulmonary hypertension, newly diagnosed in the current treatment era, when investigators can use the contributions of genomics and clinical imaging tests to further understand the disease. This registry should advance knowledge in the field both from a molecular (genomic and proteomic) and from a clinical phenotype standpoint.

The purpose of this study is to evaluate the safety, tolerability, and effectiveness of ubenimex in patients who have pulmonary arterial hypertension, to improve exercise capacity and delay clinical worsening.

The International Balloon Pulmonary Angioplasty (BPA) Registry is a prospective, multi-center, long-term observational project. Scheduled to start data collection in Q4 2017, the registry will run for approximately four years with a follow-up time for each patient of at least two years. Its primary objective is to investigate the efficacy and safety of BPA intervention in patients with chronic thromboembolic pulmonary hypertension (CTEPH) not amenable to pulmonary endarterectomy (PEA).

The purpose of this study is to demonstrate the safety and tolerability of repeated doses of L606 in patients with PAH switching from a stable Tyvaso dose. The current Phase 3 study will help determine the short-term and long-term safety and tolerability of L606 in this patient population. The study will also evaluate the steady-state  pharmacokinetics (PK) of L606 as compared to Tyvaso, effects on exercise ability (6-minute walk distance [6MWD] and Borg Dyspnea Score), quality of life (QoL), and treatment satisfaction with L606 in patients with PAH.

Part 1 of the study is short-term (2-week) evaluation of L606 in patients with PAH. Part 2 of the study will ...

The purpose of this study is to demonstrate the effect of ralinepag the time to first adjudicated clinical worsening event in subjects with pulmonary arterial hypertension (PAH).Clinical worsening event is defined as 1 of the following: death, nonelective hospital admission of <24 hours="" due="" to="" worsening="" pah="" or="" rhf,="" initiation="" of="" parenteral="" or="" inhaled="" therapy,="" disease="" progression,="" or="" unsatisfactory="" long="" term="" clinical="">

The purpose of this study is to evaluate the long-term safety, tolerability, and effectiveness of ubenimex for the treatment of patients who have pulmonary arterial hypertension, to improve exercise capacity and delay clinical worsening.

The U.S. CTEPH Registry is a multicenter, observational, U.S.-based study of the clinical course and treatment of patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH), WHO Group IV Classification for Pulmonary Hypertension. The mission of the Registry will be to promote a greater understanding of the prevalence, pathophysiology, evaluation, and treatment of patients with CTEPH through shared information, education, and collaborative investigation among pulmonary hypertension (PH) centers of excellence throughout the U.S.

The purpose of this study is to establish the long-term safety of AV-101. The long-term effects of AV-101 on efficacy measures (e.g., 6MWD, NT-proBNP, cardiac echo) will also be assessed.

The primary purpose of this study is to measure and improve quality of care (including assessing differences in adherence to evidence-based guidelines and establishing benchmarks for health outcomes).

The purpose of this study is to evaluate the effects of sotatercept (MK-7962, formerly called ACE-011) treatment (plus maximum tolerated background pulmonary arterial hypertension (PAH) therapy) versus placebo (plus maximum tolerated background PAH therapy) on time to first event of all cause death, lung transplantation, or PAH worsening-related hospitalization of ≥24 hours, in participants with World Health Organization (WHO) functional class (FC) III or FC IV PAH at high risk of mortality.

The aim of this study is to evaluate the effects of a home-based program with health coaching of dyspnea and exercieses in subjects with pulmonary arterial hypertension (PAH).

Pulmonary rehabiltation has been shown to be an effective treatment for patients with chronic obstructive pulmonary disease, however there is currently a paucity of evidence to extrapolate this informaiton with patients with pulmonary arterial hypertension (PAH). In addition, there are no options outside of center-based pulmonary rehabilitation which have been proposed as an effective treatment option for patients with PAH.

The primary purpose of this study is to determine the impact of two interventions against insulin resistance on the composite endpoint of 10% improvement in baseline six minute walk distance or improvement in WHO functional class.

The specific purpose of this study is to establish a national biorepository of biological samples and genetic data of patients with the World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH).   The function of this biorepository is to collect the samples from the patients, generate the proposed genetic data, and widely promote and distribute the samples/data to the scientific community.  Investigators requesting samples will submit requests and proposed uses of the samples which will be reviewed by a committee comprised of investigators from several of the enrolling centers.

This study will compare the efficacy, safety, and tolerability of three doses of GS-4997 to placebo in adults with pulmonary arterial hypertension (PAH). The study will consist of a 24-week placebo-controlled treatment period and a long-term GS-4997 treatment period. Participants completing the 24-week placebo-controlled period will be eligible to receive active treatment with GS-4997 in the long-term treatment period.

The PHAR is a multicenter, prospective registry of newly evaluated patients at PHCCs in the United States who have either PAH or CTEPH.  Baseline information will be collected at the time of initial evaluation at the PHCC with follow-up data collected at approximately 6-month intervals.

This study is being conducted to assess the long-term safety, tolerability, and efficacy of sotatercept (MK-7962, formerly called ACE-011) in participants with Pulmonary Arterial Hypertension (PAH). This open-label, long-term follow-up (LTFU) study is supported by data from the PULSAR study (Phase 2, NCT03496207) in which treatment with sotatercept resulted in hemodynamic and functional improvements in the study participants, including those receiving maximal PAH therapy with double/triple drug combinations and intravenous prostacyclin.

The primary objective of this open-label, LTFU study is to evaluate the long-term safety and tolerability of sotatercept when added to background PAH therapy in adult participants with PAH who ...

The purposed of this study are to investigate whether structured exercise training improves cardiac and pulmonary haemodynamic function, exercise capacity, and short-term clinical outcomes in heart failure (HF) patients with Pulmonary Hypertension (PH), and to develop a multivariable exercise pulmonary gas exchange ‘score’ that accurately differentiates HF patients with pre- and post-capillary PH (CpcPH) from HF patients with isolated post-capillary PH (IpcPH).

The primary purpose of this study is to demonstrate the safety and effectiveness of ozanimod compared to placebo on the maintenance of clinical remission and endoscopic response.

The purpose of this study is to provide continued access to TNX-103 and to assess long-term safety of TNX-103 in patients with PH-HFpEF.

The purpose of this study is to determine the association between patient voice signal characteristics and the presence and severity of pulmonary hypertension in patients referred for elective clinically indicated echocardiography for at Mayo Clinic MN.

The purpose of this study is to evaluate the effectiveness and safety of sotatercept versus placebo in adults with Cpc-PH due to HFpEF. Effectiveness is measured by change from baseline in pulmonary vascular resistance (PVR, primary endpoint) and 6-minute walk distance (6MWD, key secondary endpoint).

The purpose of this study is to evaluate the effectiveness and safety of intermittent levosimendan compared with placebo in hemodynamic improvement with exercise in PH-HFpEF subjects.

The primary purpose of this study is to administer the PAH SYMPACT questionnaire in 200 consecutive eligible patients attending the Mayo Clinic pulmonary hypertension clinic. An additional patient satisfaction survey will be administered for patients that completed a palliative care consult.

The purpose of this study is to assess the safety and efffectiveness of pulsed inhaled nitric oxide (iNO) in subjects at risk for pulmonary hypertension associated with pulmonary fibrosis on long term oxygen therapy.

The purpose of this study is to evaluate the safety, performance, and initial clinical efficacy of the Rivet PVS therapy in patients with symptomatic pulmonary hypertension.

The aims of this study are to establish a registry and biorepository of patients with PH-CKD, and to identify clinical risk factors and biomarkers associated with PH-CKD and different hemodynamic phenotypes. 

The purpose of this open-label study is to evaluate the safety of continued therapy with inhaled treprostinil in participants who have completed Study RIN-PH-304 (NCT03496623). This study hypothesizes that long-term safety findings will be similar to those observed in the randomized, placebo-controlled, double-blind, adaptive study 'A Phase 3, Randomized, Placebo-controlled, Double-blind, Adaptive Study to Evaluate the  and Efficacy of Inhaled Treprostinil in Patients with Pulmonary Hypertension due to Chronic Obstructive Pulmonary Disease (PH-COPD)(RIN-PH-304).

The enormous and rapidly growing burden of Heart Failure with Preserved Ejection Fraction (HFpEF) has led to a need to understand the pathogenesis and treatment options for this morbid disease. Recent research from the investigator's group and others have shown that pulmonary hypertension (PH) is highly prevalent in HFpEF, and right ventricular (RV) dysfunction is present in both early and advanced stages of HFpEF.

These abnormalities in the RV and pulmonary vasculature are coupled with limitations in pulmonary vasodilation during exercise. There are no therapies directly targeted at the pulmonary vasculature that have been clearly shown to be effective ...

This study will evaluate the efficacy of TNX-103 (oral levosimendan) compared with placebo in subjects with PH-HFpEF as measured by the change in 6-Minute Walk Distance (6 MWD; Day 1 to Week 12).

The purpose of this study is to assess the efficacy and safety of the study drug, levosimendan (given orally), compared to placebo in participants with pulmonary hypertension with heart failure with preserved left ventricular ejection fraction (PH-HFpEF) as measured by the change in 6-Minute Walk Distance.

This study is a multi-center, prospective, randomized, double blind, placebo-controlled clinical trial. Subjects in the study will be adults with New York Heart Association (NYHA) Class II-IV heart failure (HF) due to left ventricular systolic dysfunction (LVSD), left ventricular ejection fraction (LVEF) <0.40, and secondary pulmonary hypertension (PH). The purpose of the study is to evaluate the safety, effectiveness, and effects of tadalafil compared to placebo on the subjects' functional capacity / quality of life.

24-week study to evaluate the efficacy and safety of macitentan for the treatment of portopulmonary hypertension.

The purpose of this study is to characterize pulmonary hemodynamic profiles, with a focus on PVR subtypes, in patients with left-sided valvular heart disease undergoing aortic or mitral valve intervention,  and to identify patterns predicting reversibility of TR and RV dysfunction.

SYMPHONY is prospective, multi-center, open-label, single-arm, Phase 3b psychometric validation study of the PAH-SYMPACT, a new quality of life questionnaire for patients with pulmonary arterial hypertension. Patients will be in the study for 5 1/2 months, 4 months of which they will receive macitentan, 10 mg, once daily.

The primary objectives are to demonstrate the final content validity of the PAH SYMPACT instrument, to demonstrate the psychometric characteristics of reliability and construct validity of the PAH-SYMPACT instrument, and to demonstrate the ability of the PAH SYMPACT instrument to detect change. The secondary objective is to assess the safety of ...

The purpose of this study is to determine if certain genes, hormones, or other factors predict the risk of developing lung vessel disease in patients with liver disease and whether they determine outcome.

This is a prospective, non-interventional, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD).

The purpose of this study is toexamine whether increased sphingosine 1 phosphate : ceramide ratio and circulating bile acids are associated with HPS in patients with advanced liver disease.

The purpose of this study is to assess whether our not the percentage of pulmonary related vascular structures (PRVS) determined by the quantitative CT (Q-CT) tool CALIPER is a novel measure that can be used for the early detection of pulmonary vascular remodeling in patients with SSc.  pulmonary related vascular structures

We propose a pilot study to assess safety and benefit of renal artery ablation at the time of planned atrial fibrillation ablation.

A surveillance of respiratory tract related adverse events in patients treated with Tyvaso® (treprostinil) Inhalation Solution versus other FDA approved therapies.

Systolic Pressure Intervention Trial (SPRINT) is a large scale randomized trial of ~ 9250 adults aged 50 years or older with high cardiovascular risk sponsored by NIH. The study is designed to recruit 45% of the study population with Chronic Kidney Disease (CKD). The trial will test the effects of low systolic blood pressure (SBP) goal of < 120 mm Hg versus the standard goal of < 140 mm Hg on the primary composite of cardiovascular events and death. One of the pre-specified secondary outcome is the progression of kidney disease. In this ancillary named SPRINT - Factors affecting Atherosclerosis ...

The purpose is to study the correlation of right ventricular strain by tissue Doppler and Tricuspid Annular Plane Systolic Excursion (TAPSE) in patients without a tricuspid regurgitation jet with the presence and severity of pulmonary artery hypertension diagnosed by right heart catheterization. The primary aim is to determine the correlation of other echocardiographic, laboratory and clinical findings in patients without a tricuspid regurgitation jet with the presence and severity of pulmonary artery hypertension diagnosed by right heart catheterization. As such, one goal is to potentially create a predictive score with adequate sensitivity to determine which patients without a tricuspid regurgitation ...

The purpose of  this study is to determine the usefulness of palliative care consultations by a specialty physician and a pulmonologist for patients who have advanced lung disease.

The purpose of this study is to determine if vasodilators for the lungs can decrease liver stiffness, shown by Magnetic resonance elastography (MRE).  The study also aims to identify any clinical, laboratory, echocardiographic, imaging, and hemodynamic parameters that connect with improved outcomes of cardiac and liver status after starting the pulmonary vasodilators.

The eventual goal of this study is to identify genetic and proteomic markers that may influence susceptibility to vascular diseases including peripheral arterial disease, pulmonary hypertension, carotid artery stenosis, arterial aneurysmal disease, fibromuscular dysplasia, congenital heart disease and other less common diseases that affect the blood vessels.

The purpose of this study is to evaluate the safety, pharmacokinetics (PK), pharmacodynamics (PD), and biomarkers of inhaled GB002 in adults with World Health Organization (WHO) Group 1 Pulmonary Arterial Hypertension (PAH).

The purpose of this study is to evaluate the effietiveness of remdesivir (RDV) in reducing the rate of of all-cause medically attended visits (MAVs; medical visits attended in person by the participant and a health care professional) or death in non-hospitalized participants with early stage coronavirus disease 2019 (COVID-19) and to evaluate the safety of RDV administered in an outpatient setting.