Active clinical trials for "Familial Primary Pulmonary Hypertension"

Pulmonary arterial hypertension (PAH) involves the narrowing of blood vessels connected to and within the lungs. Focal adhesion kinase (FAK) is a focal adhesion-associated protein kinase involved in cellular adhesion and spreading processes which leads to stiffening and thickening of blood vessels in lungs. This further increases the blood pressure within the lungs and impairs their blood flow. This study aims to demonstrate biodistribution of FAK inhibitor, GSK2256098, in cardiopulmonary sites i.e. heart and lung tissues. The uptake of 11C-radiolabelled GSK2256098 within the lung and / or heart of healthy subjects and idiopathic pulmonary arterial hypertension (iPAH) patients will be assessed using Positron Emission Tomography (PET). Based on study results the decision as to whether a follow-on phase 2 trial should be initiated to investigate the therapeutic utility of GSK2256098 in iPAH will be decided. Sufficient number of subjects will be screened (up to 30 days prior to scan) to enrol 12 healthy subjects and 12 PAH patients in the study. Enrolled subjects will receive a microdose of [11C]-GSK2256098 on a scanning day which will be followed by the follow-up visit (2-4 weeks post-last dose).

Pulmonary arterial hypertension (PAH) is progressive life limiting disease with a median survival of less than 3 years without treatment. Current drug trials in PAH commonly use simple tests for example the 6-minute walk test, blood tests such as N-terminal pro-brain-type natriuretic peptide (NT-pro-BNP) and BNP, and haemodynamic measures such as PAP and PVR obtained by RHC as endpoints. These tests are surrogate markers of disease severity in patients with pulmonary hypertension. There is now evidence suggesting that magnetic resonance imaging (MRI) may be helpful in the follow up of patients with PAH with high accuracy for the detection of treatment failure, this is because MRI can track changes occurring in the heart by direct visualisation of cardiopulmonary morphology and function, an advantage over existing methods. However, the reproducible of MRI measurements in patients with PAH is not known, and the comparative repeatability of MRI in relation to traditional candidate endpoints such as walk tests and blood tests used in drug trials is not known.

Prevalence of musculoskeletal pain and its impact of quality of life and functional exercise capacity will be evaluated in patients with pulmonary arterial hypertension.

Patients with pulmonary arterial hypertension(PAH) suffer from chronic shortness of breath, and have impaired survival related to progressive right ventricular failure. Abnormal vasoreactivity to nitric oxide(NO) plays a role in the pathophysiology of PAH. Phosphodiesterase Type 5 Inhibitor (PDE5 inhibitors) sildenafil have been shown to be beneficial in PAH, but extent of benefit is variable.

Assess tolerability, transition methods and clinical effects of transition from sildenafil (Revatio) to tadalafil (Adcirca) for treatment of pulmonary arterial hypertension.

The Ventavis® (iloprost) Registry is a multicenter, observational, U.S.-based study that longitudinally follows patients with pulmonary arterial hypertension (PAH) who have been receiving therapy with Ventavis® for at least 3 months. Patients diagnosed with WHO Group I PAH who are on a stable regimen of commercial Ventavis® will be followed for a maximum of 2 years from the time of enrollment. Data will be collected via patient interview and review of the medical record. Quarterly data collection will include capture of medications and Ventavis® adherence data.

Pulmonary arterial hypertension(PAH) is associated with the development of right heart failure. In the setting of heart failure, the heart shifts to increasing dependence on glucose metabolism. In this study, the investigators will perform cardiac positron emission tomography/magnetic resonance imaging (PET/MRI) scans to measure glucose metabolism in the heart before and after initiation of pulmonary vasodilator therapy for pulmonary arterial hypertension.

The purpose of this study is to see how inhaled treprostinil sodium (Tyvaso) affects the amount of air and blood that reach the alveoli, or tiy air sacs, in the lungs of patients with Group 1 Pulmonary Arterial Hypertension with concomitant Chronic Obstructive Pulmonary Disease (COPD).

A two-stage prospective observational cohort study in scleroderma patients to evaluate screening tests and the incidence of pulmonary arterial hypertension and pulmonary hypertension

Right heart failure is the main cause of morbi-mortality in patients with pulmonary hypertension (PH), including patients of chronic thrombo-embolic pulmonary hypertension (CTEPH) or pulmonary arterial hypertension (PAH) etiologies. Endarterectomy is an effective treatment for patients with CTEPH to lower pulmonary pressure. Evidence of postoperative right heart remodeling are contrasted according to the studies and determinants of right heart failure are still unclear. Similarly, few evidence exists on right ventricular remodeling after bilateral lung transplantation for patients with pulmonary arterial hypertension (PAH). Recent evidence have supported the role of inflammation and immunity in the pathophysiology of PAH . While several cytokines have been shown to predict survival , little is known on the implication of inflammation and immunity in postoperative Right Ventricular failure in patients with PAH. • The specific translational goal of this current project is to elucidate the role of immune biomarkers in 6 months postoperative right heart adverse remodeling in patients with CTEPH or PAH. We speculate that selected immune biomarkers (such as CXCL9, interleukin -18 or interferon) and growth factors (such as HGF) are correlated with mid-term postoperative right heart failure. All consecutive adults with either CTEPH referred to our center for endarterectomy, or PAH referred for lung transplantation, will be included, aiming for 150 CTEPH and 50 PAH. After inclusion, patients will undergo assessment of right heart dimensions and function by cardiac magnetic resonance imaging (MRI, including 4-Dimensions blood flow sequences) and 2D and 3D trans-thoracic echocardiography (TTE), as well as immune panel analysis. All patients will undergo as part of routine care right heart catheterization within a week after TTE and MRI imaging. On the day of surgery, pulmonary pressure will be measured by right heart catheterization monitoring (as part of routine care) in order to estimate the drop of pressure and to adjust for the extent of endarterectomy for patients with CTEPH. TTE will also be performed on the day of surgery if possible. At 7 days post-endarterectomy or transplant, clinical outcomes will be collected and peripheral blood will be collected. Patients will be prospectively follow-up for 6 months. Death, need for reintervention, duration of vasopressor after surgery and number, duration and cause of readmission will be recorded. At 6 months after surgery, all survivors will undergo the same biological sampling, as well as an 4D MRI and a 2D and 3D TTE. Data of right heart catheterization at 6 months (as part of routine care) will be collected as well.