Active clinical trials for "Pulmonary Arterial Hypertension"

The aim of the study is to determine relevant hemodynamic parameters for the diagnostics of pulmonary arterial hypertension (PAH) by dynamic contrast enhanced dual-energy CT (DE-CT). In this prospective study the investigators validate DE-CT results of patients against hemodynamic parameters from right heart catheterisation and control the results by other clinical investigations. The investigators expect that using this non-invasive method, parameters relevant for the diagnosis of the patients with PAH, like pulmonary blood volume, blood flow and perfusion heterogeneity, can be determined.

The aim of the study is to determine relevant hemodynamic parameters for the diagnostics of pulmonary arterial hypertension (PAH) by dynamic contrast enhanced dual-energy CT (DE-CT). In this pilot study the investigators investigate patients who underwent right heart catheterisation with DE-CT to determine hemodynamic parameters and control the results by other clinical investigations. The investigators expect that using this non-invasive method, parameters relevant for the diagnosis of the patients with PAH, like pulmonary blood volume, blood flow and perfusion heterogeneity, can be determined.

Pulmonary artery hypertension (PAH) is a rare, severe disease, characterized by a progressive increase in pulmonary vascular resistance ultimately leading to right ventricular (RV) failure and premature death. PAH may be idiopathic (IPAH) or may be also related to various conditions like portal hypertension, HIV infection, left to right shunt, connective tissue diseases such as scleroderma (PAHSSc). Symptoms include dyspnea and fatigue resulting in restricted exercise capacity and poor quality of life. The therapies currently approved have been shown to improve survival. Indeed, recent studies described a three year survival higher than 80%. This improved survival is associated with major challenges for clinicians as most patients remain with limited exercise capacity and poor quality of life. A clear understanding of exercise physiopathology is thus mandatory to specifically address mechanisms responsible for this exercise limitation and eventually improve patients' management. In order to better characterize the exercise physiopathology in PAH, the general objective of this research is to systematically examine blood flow distribution and limb muscles microcirculation at rest and during submaximal exercise in PAH.

The purpose of this study is to determine the clinical, functional and hemodynamic response after six months of sildenafil 50 mg TID in patients with pulmonary arterial hypertension.

The objectives of the current study are to identify and evaluate new prognostic non-invasive and serological markers in patients with pulmonary hypertension. The focus will be on L-arginine metabolism and to clarify its influence on endothelial function.

An echocardiogram using Definity contrast can provide the same information as the standard right heart catheterization.

Pulmonary artery hypertension (PAH) is a chronic and progressive disease that affects 15 persons per million. Although current therapy has improve disease prognosis, PAH still has a poor survival, with a median survival of 2.8 years after diagnosis. In the last few years new key elements in PAH pathogenesis have been discovered, such as the role of metabolism in disease onset and progression. In fact, PAH pulmonary smooth muscle cells switch into a glycolytic phenotype which resembles the metabolism of cancer cells. The investigators hypothesis is that "fatty acid oxidation inhibition reverts the PAH adverse phenotype by restoring mitochondrial function and morphology, decreasing proliferation and restoring apoptosis susceptibility in pulmonary smooth muscle cells "

This study will examine the effects of add-on sildenafil to bosentan monotherapy in patients with Pulmonary Arterial Hypertension. Patients on bosentan monotherapy will be followed every 6 months to assess if they have met the pre-defined treatment goals. If a patient fails to achieve these treatment goals or fails to maintain them, sildenafil will be added to their existing bosentan monotherapy. Patients will be assessed 6 months after start of combination therapy for changes in 6MWT, Borg dyspnea scale, WHO functional class, quality of life.

The study is to assess if sitaxsentan and ritonavir will affect the blood levels of each other when coadministered.

This study will assess how rifampin will affect the blood levels of sitaxsentan. Safety of sitaxsentan given alone and with rifampin will also be assessed.