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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…

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-…

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 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).

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…

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…

Exercise-induced left ventricular diastolic dysfunction in COPD patients with a symptomatic limitation to exercise

The primary aim of this observational study is to determine the occurrence of exercise-induced left ventricular diastolic dysfunction (LV-DD) in symptomatic COPD patients with a normal echocardiography at rest. And, secondly, to study 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 Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

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 longterm safety and tolerability of oral BAY 63-2521 in patients…

Registry of Sarcoidosis Associated Pulmonary Hypertension (RESAPH)

We propose to develop a multicenter registry of sarcoidosis associated pulmonary hypertension (SAPH). With this registry, we will characterize the demographics, clinical course, hemodynamics, pulmonary physiology, and disease management of…

MRI analysis of PAVM's

To demonstrate that MRI is as good in the detection of clinically relevant PAVM as the CT.

AMB116457: An open-label extension study of the long-term safety, tolerability and efficacy of ambrisentan in subjects with inoperable chronic thromboembolic pulmonary hypertension (CTEPH)

Primary: to assess the longterm safety and tolerability of ambrisentan 5mg in subjects with inoperable CTEPH. Secondary: to collect supportive efficacy data.

An open-label phase IIIb study of riociguat in patients with in-operable CTEPH, or recurrent or persisting PH after surgical treatment who are not satisfactorily treated and cannot participate in any other CTEPH trial

Objectives of the study are to assess safety, tolerability and clinical effects of riociguat and provide access to inoperable patients for a condition (CTEPH) with unmet medical need.

An Open-Label Extension Trial of UT-15C SR in Subjects with Pulmonary Arterial Hypertension

To provide, or continue to provide, UT-15C SR for eligible subjects who participated in protocols TDE-PH-301 or TDE-PH-302 or TDE-PH-308 or additional UC-15C SR clinical protocols.To assess the long-term safety of UT-15C SR in these subjects through…

An open-label extension of study AC-066A301 investigating the safety and tolerability of ACT-385781A in patients with pulmonary arterial hypertension (PAH)

To assess safety and tolerability of EFI patients with PAH.