[18F]FES PET/CT in PAH
In this study positron emission tomography (PET/CT) imaging will be used to evaluate evaluation of estrogen receptor heterogeneity and functionality in Pulmonary Arterial Hypertension (PAH) using the investigational radiotracer [18F]fluoroestradiol (FES).
3D Echo Evaluation of the Ventricles in Sclero Associated Severe PAH
The aim of the study is to document the left and right ventricular function in patients with sclerodermia associated pulmonal arterial hypertension via 3D echocardiography. The results of this study should help to generate hypothesis for further future studies. The primary hypothesis is that the increased use of modern image guided methods could provide essential aspects for the follow up.
3D Imaging: Prognostic Role in Pulmonary Arterial Hypertension
Pulmonary hypertension is a rare and severe disease, affecting a young population. Survival is very poor and has been closely related to right ventricular dysfunction. Current prognostic equations rely mostly on right heart catheterization data. The identification of simple echocardiographic prognostic factor is urgently needed. It could help identifying with a non invasive method, high risk patients who could benefit from an intensive specific therapy. 3D right ventricular imaging is a new echocardiographic tool which provides RV volumic analysis, RV ejection fraction, overcoming the classical limits of 2D ultrasound. The aim of this...
4DFlow Magnetic Resonance Imaging in Patients With Pulmonary Hypertension Associated With Congenital Heart Disease
Congenital heart disease is the most common congenital anomaly. The life expectancy of children with congenital heart disease has increased considerably in recent years. Nevertheless, the evolution of these patients is marked by an increased risk of complications. Arrhythmias, heart failure, pulmonary arterial hypertension (PAH) and endocarditis may be promoted by the absence or delay of management in childhood, by residual lesions or post-operative cardiac scars and by the presence of prosthetic materials. PAH is a common complication of congenital heart disease, especially in non-operated shunts. PAH corresponds to an increase in...
ABI-009, an mTOR Inhibitor, for Patients With Severe Pulmonary Arterial Hypertension
mTOR activation has been shown to be relevant in the development and progression of pulmonary hypertension. Inhibition of mTOR has been shown to reverse or regress pulmonary hypertension in animal models. ABI-009 is an albumin-bound mTOR inhibitor with improved penetration in lung tissue.
A Clinical Study in France to Investigate the Long-term Safety of the Drug Macitentan in Patients With Pulmonary Arterial Hypertension Who Were Previously Treated With Macitentan in Clinical Studies.
The aim of the trial is to study the long-term safety of macitentan and to provide continued treatment with macitentan to patients with pulmonary arterial hypertension (PAH) who were previously treated with macitentan in clinical studies.
A Clinical Study of to Confirm the Doses of Selexipag in Children With Pulmonary Arterial Hypertension
Selexipag is available for the treatment of pulmonary arterial hypertension (PAH) in adults in various countries. The efficacy of selexipag to delay disease progression was shown in a previous pivotal study conducted in 1156 adult patients with PAH. Given the similarities in the functional changes of PAH in children and adults, it is expected that children suffering from PAH could benefit from treatment with selexipag. The aim of the present study is to confirm the doses of selexipag to be used in pediatric patients with PAH older than 2 years. To fulfill this aim, blood levels of selexipag (pharmacokinetic assessments) as well as the ...
A Clinical Study to Find Out if Macitentan is Effective and Safe in Japanese Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
The endothelin receptor antagonist macitentan showed significant improvement compared with placebo in pulmonary vascular resistance (PVR) and 6-minute walking distance (6MWD) in inoperable CTEPH patients in the phase II MERIT-1 trial (AC-055E201, NCT02021292). However, in the MERIT-1 trial Japanese patients were not included. Therefore, in line with Japan's medical environment, this phase III study is to confirm the efficacy and safety of macitentan in Japanese CTEPH patients.
ACT-293987 in Pulmonary Arterial Hypertension
Long-term, single-arm, multicenter, open-label extension, Phase 3 study, to evaluate the safety and tolerability of ACT-293987 in patients with PAH who participated in the double-blind study AC-065A302 (GRIPHON)
Actigraphy in Pediatric Pulmonary Hypertension
Physical activity is an important factor in understanding how diseases can affect a child. Decreases in physical activity are sometimes the first thing that happens before a child is diagnosed with a disease. When a child sees their doctor, that visit reflects a single point in time. It does not capture how a child feels during the days in between visits to their doctor. This study plans to use special monitors called actigraphs to collect information about physical activity in children with a type of disease called pulmonary hypertension. This study will measure how active these children are and compare their activity to clinical ...
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