Non-invasive Pulmonary Artery Prediction (ADOPTS)
Heart FailurePulmonary Arterial HypertensionA proprietary machine-learning algorithm has been developed to model continuous pulmonary artery pressure (PAP), a physiologic marker of cardiopulmonary function. The algorithm was developed from PAP recordings obtained during invasive right heart catheterization. The study will evaluate whether this algorithm can perform as well when embedded into a non-invasive wearable device that records EKG, heart sounds, and thoracic impedance has yet to be established.
Hypotonic Treprostinil Subcutaneous Infusion for Control of Treprostinil Related Site Pain
Pulmonary Arterial HypertensionThe investigators will test whether hypotonic (diluted) remodulin solution causes less pain than the eutonic (undiluted) solution supplied by the manufacturer.
Assessment of Right Ventricular Function by Cardiac MRI in Patients With Pulmonary Arterial Hypertension...
Pulmonary Arterial HypertensionPulmonary hypertension (PH) is a pathophysiological condition defined by an increase in pulmonary arterial pressure above 20mmHg, which encompasses many very dissimilar conditions. Right ventricular function is the major determinant of survival in these patients. Currently, right ventricular function is estimated by trans-thoracic echocardiography via the measurement of standardised parameters. However, cardiac magnetic resonance imaging is now the reference technique for non-invasive quantification of volumes, mass, function of the right ventricle but can also be useful for the consideration of the pulmonary circulation. Thus, indices of function can be extracted and it plays an increasing role in the prognostic evaluation of the right heart function at diagnosis and at re-evaluation under treatment of pulmonary arterial hypertension. This work will initially focus on the assessment of right ventricular myocardial work by echocardiography and cardiac magnetic resonance imaging in comparison with invasive haemodynamic data.
Study of PAH Subjects With LTOT Use That Have Demonstrated Improved Exercise Tolerance With the...
Pulmonary Arterial HypertensionStudy of PAH Subjects with LTOT Use that have Demonstrated Improved Exercise Tolerance with the use of Inhaled Nitric Oxide
Albuterol and Dynamic Hyperinflation in Idiopathic Pulmonary Arterial Hypertension
Idiopathic Pulmonary Arterial Hypertension.The purpose of this study is to determine if dynamic hyperinflation seen in patients with idiopathic pulmonary artery hypertension (iPAH) improves with albuterol therapy.
Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis and Treatment With Bosentan...
Pulmonary Arterial HypertensionIdiopathic Pulmonary FibrosisPulmonary Arterial Hypertension (PAH) in the setting of Idiopathic Pulmonary Fibrosis(IPF)is a risk factor for morbidity and mortality in the peri-lung transplant(LT) setting. Currently there is no significant data to support the use of pulmonary vasodilators for PAH in the setting of interstitial lung disease such as IPF. The majority of IPF patients have PAH either at rest or during exercise. The study hypothesis is that bosentan may improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH.
This Study Tests the Safety of Inhaled BAY1237592, How the Drug is Tolerated and How it Effects...
HypertensionPulmonaryIn this trial the effects of the inhaled drug BAY1237592 will be studied in patients with high blood pressure in the pulmonary blood vessels due to Pulmonary Arterial Hypertension (PAH) and due to Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Pulmonary hypertension is characterized by the elevation of pressure in the pulmonary arteries (PAP) and of the pulmonary vascular resistance (PVR) leading to increased workload of the right chamber of the heart to eject blood against this elevated resistance. The goal of this study is to measure the safety and tolerability of the drug as well as the reduction of the PVR at different doses In Part A patients without specific treatment for PH (untreated patients) will be tested. In Part B also patients stably pre-treated with specific PH drugs will be studied in combination with the new inhaled drug
Role of Endothelin-A (ETA) and Endothelin-B (ETB) Receptors in the Vasodilatory Response to Endothelin-3...
Pulmonary Arterial HypertensionVasodilation1 moreEndothelin-1 (ET-1) has been linked to a number of conditions including pulmonary arterial hypertension (PAH). ET-1 acts via 2 receptors, ETA and ETB. The ET-1 receptor blockers bosentan and sitaxsentan have been shown to be beneficial in patients with PAH. Bosentan blocks both ETA and ETB receptors. Sitaxsentan selectively blocks ETA receptors. Theoretically, selective ETA blockade may be associated with greater vasodilation and clearance of ET-1 by leaving the ETB receptor unblocked. This has not been directly studied in humans. We aim to investigate the endothelial ETB-mediated vascular responses between bosentan and sitaxsentan by using a ETB selective agonist (ET-3). We hypothesise that at clinically relevant doses: Bosentan will show evidence of ETB receptor blockade compared to sitaxsentan and placebo. These effects will be confirmed by 2 functional markers of ETB receptor antagonism: plasma ET-1 (a very sensitive, but not necessarily clinically relevant marker), and the forearm vasodilator response to ET-3.
Safety, Tolerability, & Pharmacokinetics Study of Single & Multiple Inhaled Doses of Imatinib Inhalation...
Pulmonary Arterial HypertensionThis is a randomised, double-blind, placebo-controlled, dose escalation study to evaluate the safety, tolerability, and PK of single and multiple inhaled doses of imatinib inhalation solution (AER-901) in healthy adult volunteers. This study consists of 3 parts and an optional fourth part.
Effect of Selexipag on Daily Life Physical Activity of Patients With Pulmonary Arterial Hypertension....
Pulmonary Arterial HypertensionThe primary objective of this study is to evaluate the effect of selexipag on the physical activity of patients with pulmonary arterial hypertension (PAH) in their daily life, by using a wearable wrist device (actigraph). The actigraph will collect data on daily life physical activity in the patient's real environment. In addition, the PAH symptoms and their impacts will be assessed by using an electronic patient reported outcome measure in the patient's real environment. Patients will be assigned randomly to either selexipag or placebo.