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An extension study to QTI571A2301 to evaluate the longterm safety, tolerability and efficacy of oral QTI571 (imatinib) in the treatment of severe pulmonary arterial hypertension.

Primary: To evaluate the long-term safety and tolerability of QTI571.Secondary: * Continue to evaluate the long-term efficacy of QTI571 as measured by the change in6MWD from baseline.* Continue to assess time to clinical worsening (TTCW) endpoints…

An open-label, long term extension study for treatment of pulmonary arterial hypertension in paediatric patients aged 8 years up to 18 years who have participated in AMB112529 and in whom continued treatment with ambrisentan is desired (AMB114588)

Primary: Longterm safety and tolerability of 2 dosages of ambrisentan. Secundary: Supportive efficacy data.

Myocardial function, oxygen consumption and efficiency assessment in the right ventricle of patients with idiophathic pulmonary hypertrophy using positron emission tomography.

The study investigates the role of the RV efficiency in the deterioration of RV function to failure in PH (measured with stroke volume index as the parameter of RV function); efficiency will be related to MVO2, alterations in substrate metabolism…

Structural and Functional Microcirculatory Aspects of Patients with (suspected) Connective Tissue Disease and Pulmonary Arterial Hypertension

The proposed project focuses on: a) implementation of an improved, standardized method for performing quantitative nailfold capillaroscopy to visualize morphological patterns and evaluate its diagnostic use in patients with (possible) CTD and…

A randomized, open label study comparing safety and efficacy parameters for a high and a low dose of ambrisentan (adjusted for body weight) for the treatment of pulmonary arterial hypertension in paediatric patients aged 8 years up to 18 years (AMB112529)

Primary: Safety and tolerability of 2 dosages of ambrisentan. Secundary: Efficacy and PK.

The Effects of Epidermal Growth Factor Receptor (EGFR) Inhibition on Pulmonary Arterial Hypertension Associated with Systemic Sclerosis
A phase II controlled open-label safety and efficacy study.

The first objective is to evaluate the safety of cetuximab in patients with scleroderma associated PAH. The secondary objective is to assess efficacy.

Gallium-67 scintigraphy in right ventricular disease

To study whether inflammmation plays a role in patients with RV disease, and can be detected with Gallium-67 scintigraphy

Restoration of cardiac function and regional pulmonary blood flow in chronic thromboembolic pulmonary hypertension after pulmonary endarterectomy assessed during exercise by magnetic resonance imaging

To assess, by use of MRI measurements during exercise, the level of restoration of cardiac function, and to study whether excercise limitation in CTEPH patients after a hemodynamically succesful PEA is caused by an impaired RV stroke volume response…

Exercise training in patients with idiopathic Pulmonary Arterial Hypertension

1. Does exercise training improve muscle function and exercise tolerance in patients with IPAH?2. Does exercise training in IPAH result in morphological and biochemical changes of the quadriceps muscle?3. Does exercise training in IPAH result in…

Pulmonary hypertension in sarcoidosis: optimizing the diagnostic approach

The first objective of the study is to predict the prevalence of PH in sarcoidosis with pulmonary involvement, and to optimize the diagnostic approach in screening for PH associated sarcoidosis using the RHC as gold standard (Galie, 2009). The…

Long-term single-arm open-label phase 3 study, to assess the safety and tolerability of selexipag (ACT-293987) in patients with pulmonary arterial hypertension

To assess the long-term safety and tolerability of selexipag (ACT-293987) in patients with pulmonary arterial hypertension (PAH).

Towards a better understanding of patients in the *TWILIGHT zone* of Pulmonary Hypertension (OPTIEK2)

The main objective of the current study is to evaluate which patients in the twilight zone of PH will benefit from PAH specific therapy. We will assess the (early) response to PAH specific treatment and determine potential predictors of adverse…

Long term, multicenter, single-arm, open-label extension study of the MERIT-1 study, to assess the safety, tolerability and efficacy of macitentan in subjects with inoperable chronic thromboembolic pulmonary hypertension (CTEPH)

Safety objective:• To evaluate the long-term safety and tolerability of macitentan 10 mg in subjects withinoperable CTEPH.Efficacy objectives:• To evaluate the long term effects of macitentan 10 mg on exercise capacity andfunctional class (FC).

Combined Pressure and Doppler Flow Velocity Assessment In Pulmonary Hypertension

In this explorative study, we aim to investigate whether MVR obtained with Doppler flow velocity, is a better predictor of adverse outcome than PVR obtained with Swan-Ganz measurements.

Heparin management and hemostatic risk evaluation in patients undergoing pulmonary thrombo-endarterectomy

To provide evidence demonstrating that ACT-guided heparin management during pulmonary thrombo-endarterectomy (PTE) involving cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA) will result in inadequate heparinization and…

Resmon Pro Diary Pilot Study - Evaluation of Vivisol Service to remotely prevent COPD exacerbations

This pilot does not aim to demonstrate the effectiveness of the system. The effectiveness of the system has been demonstrated in the recently published study: Chromed study, a multi-center Randomized Controlled Study.This pilot aims to obtain…

A Study Evaluating the Long-term Efficacy and Safety of Ralinepag in Subjects with PAH via an Open-label Extension (ADVANCE Extension)

This study has been transitioned to CTIS with ID 2023-509305-68-00 check the CTIS register for the current data. To evaluate the long-term safety and tolerability of ralinepag (ADP811) in subjects who participated in a preceding Phase 2 or Phase 3…

Prevalence of asymptomatic deep vein thrombosis in COVID-19 patients admitted to the ward

To establish a point prevalence of asymptomatic proximal lower extremity DVT in patients admitted to the ward with COVID-19.

Safety and Efficacy of Low Molecular Weight Heparin for 72 Hours Followed by Dabigatran for the Treatment of Acute Intermediate-Risk Pulmonary Embolism

The primary objective is to determine whether treatment of acute intermediate-risk PE (as defined by the inclusion and exclusion criteria) with parenteral anticoagulation for at least 72 hours after diagnosis, followed by dabigatran over 6 months,…

A phase III open-label extension study to evaluate long-term safety and efficacy of PRM-151 in patients with Idiopathic Pulmonary Fibrosis (IPF)

Study WA42294 is a Phase III open-label extension (OLE) study to assess the long-term safety, efficacy, and pharmacokinetics of PRM-151 in patients with IPF with or without concurrent treatment with pirfenidone or nintedanib. Patients with IPF who…