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Iodine Mapping using Subtraction in Pulmonary Embolism Computed Tomography versus Dual Energy Computed Tomography.

To evaluate image quality and accuracy of detection of perfusion defects associated with pulmonary pathology on iodine maps of the lung that are created by two different CT techniques: 1. A standard of care CTPA with DECT and 2. A new technique that…

Ammendment of protocol: Intravenous iron treatment in iron deficient patients with idiopathic pulmonary arterial hypertension. Title amendment: Skeletal muscle strength in healthy persons and patients with pulmonary hypertension

To evaluate the effects of intravenous iron suppletion in iron deficient PAH patients.Amendment: to compare exercise capacity and isolated muscle strength in IPAH patients and healthy controls

Patterns and processes of cognitive and behavioural changes in patients with COPD receiving smoking reduction treatment - The REDUQ II study

The REDUQ II study will be conducted to help us to complete data on the secondary endpoints of the REDUQ study (P09-22) and arrange and examine relationships occurring between and among intervention elements of the REDUQ study, behavioural and…

The role of the pulmonary vasculature in the Fontan circulation

Primary Objectives:- Determine the effect of pulmonary vasodilatation on indexed cardiac output during simulated exercise.- Characterization of structural properties of small pulmonary arteries.Secondary Objectives: - Investigate the effect of…

The effects of treatment on the right ventricular exertional contractile reserve in chronic tromboembolic pulmonary hypertension patients

The aim of this study is to evaluate the effects of treatment on the exertional contractile reserve and RV-arterial coupling during exercise in CTEPH patients. Furthermore, we evaluate the effects on the exertional contractile reserve and RV-…

Non-invasive assessment of lung remodeling in patients with idiopathic pulmonary fibrosis (IPF) and pulmonary arterial hypertension (PAH) using [11C]-Nintedanib or [18F]-FLT PET imaging.

To investigate whether enhanced expression of growth factor receptors (FGF-R, PDGF-R and VEGF-R) and increasedvascular cell proliferation in the lung of IPF or PAH patients can be assessed using [11C]-Nintedanib or [18F]-FLTPET/CT imaging.

ChanGes In Right ventricular volumes And Function Following diurEtic Treatment In Pulmonary arterial hypertension patients with decompensated right heart failure (GIRAFFE)

The primary objective of the study is to evaluate the effect of diuretics on right ventricular function and volume properties in patients with symptomatic right heart failure and PH.

A prospective, multicenter, single-arm, open-label, phase 4 study to evaluate the effects of macitentan on Right vEntricular remodeling in Pulmonary ArterIal hypeRtension assessed by cardiac magnetic resonance imaging

The primary objective of the study is to evaluate the effect of macitentan on right ventricular and hemodynamic properties in patients with symptomatic PAH.Secondary objectivesTo evaluate the safety and tolerability of macitentan in patients with…

The pressure overloaded right ventricle: a deformation imaging study

1. To characterize and compare RV myocardial structure, contraction patterns, and functional capacity of (A) PH patients (group 1 or 4, the latter are to be inoperable), (B) patients with pressure overloaded RV due to CHD and (C) healthy controls. 2…

The power and the flow: raising the bars in the treatment of pulmonary arterial hypertension

The primary goal of the study is to assess whether a goal oriented strategy to preserve/improve right ventricular function by application of upfront combination therapy will improve clinical outcome. Study questions:1. Will a goal oriented strategy…

Long-term single-arm open-label extension study of the SERAPHIN study, to assess the safety and tolerability of macitentan/ACT-064992 in patients with symptomatic pulmonary arterial hypertension

To assess the long-term safety and tolerability of macitentan in patients with symptomatic pulmonary arterial hypertension (PAH).

Exercise-induced pulmonary hypertension in fibrotic idiopathic interstitial pneumonias. Correlations with cardiopulmonary exercise testing

The primary aim of this observational study is to determine the occurrence of (exercise-induced) pulmonary hypertension (sPAP > 40 mmHg) in IIP patients with an elevated V*E/V*CO2 at AT (> 34) assessed during CPET. And, secondly to…

Intravenous Administration of In Vitro Expanded Mesenchymal Stem Cells in Patients with Pulmonary Arterial Hypertension due to Systemic Sclerosis;
A Safety and Feasibility Study

The main objective is assessment of safety and feasibility of intravenous administration of autologous, in vitro expanded, mesenchymal stem cells in patients with pulmonary arterial hypertension due to systemic sclerosis. The secondary objective is…

Imaging of Ventricular Reversed Remodeling after Double Lung Transplantation in Patients with Pulmonary Arterial Hypertension

To study remodeling patterns of the left and right ventricle in patients with pulmonary arterial hypertension who underwent lung transplantation, using cardiac magnetic resonance imaging.

It's LiFe! Innovative monitoring and personalised feedback as a self-management tool in disease management programmes for people with COPD and/or type 2 diabetes; a randomised controlled trial

The main objective of this study is to measure the effects of the monitoring and feedback tool embedded in a Self-management Support Program on physical activity. The secondary objective is to measure the effect on self-efficacy, quality of life and…

Dyspnoea in Pulmonary Arterial Hypertension: Targeting the Diaphragm muscle

PRIMARY OBJECTIVE:To determine the contractile strength and the (ultra)structure of single diaphragm muscle fibers of CTEPH patients SECONDARY OBJECTIVES:1. To determine whether diaphragm muscle fiber weakness is part of a generalized muscle…

A prospective, multicenter, open-label extension of FUTURE 3 to assess the safety, tolerability and efficacy of the pediatric formulation of bosentan two versus three times a day in children with pulmonary arterial hypertension

To evaluate the long-term safety, tolerability and efficacy of the pediatric formulation of bosentan two versus three times a day in children with pulmonary arterial hypertension (PAH).

A Multicenter, Single-arm, Open-label, Phase 3b Study to Assess the Effects of Switching From Flolan® to ACT-385781A in Patients with Pulmonary Arterial Hypertension

- To evaluate the change in cardiac hemodynamics from baseline to 3-month following switch from Flolan to EFI in patients with PAH.- To evaluate the safety and tolerability of switching from Flolan to EFI in patients with PAH.- To evaluate the…

Long-term extension, multi-centre, multi-national study to evaluate the safety and tolerability of oral BAY 63-2521 (1 mg, 1.5 mg, 2 mg, or 2.5 mg tid) in patients with symptomatic Pulmonary Arterial Hypertension (PAH).

BAY 63 2521 is a stimulator of the soluble guanylate cyclase (sGC) and is intended for the treatment of cardiovascular diseases, especially pulmonary hypertension (PH).To assess the long-term safety and tolerability of BAY 63 2521 in the treatment…

A Single-arm Open Label Study Evaluating the Impact on Lifestyle of a New Thermo-stable Formulation of Flolan in Subjects with Pulmonary Arterial Hypertension (PAH) (FLR115332)

Primary:* To describe the effect of the new thermo stable formulation of FLOLAN on quality of life in patients switching from the currently marketed FLOLAN to the new thermo stable formulation.* To determine the dose titration requirementSecondary…