Active clinical trials for "Pulmonary Arterial Hypertension"

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.

Secondary data collection study: safety and effectiveness of Revatio in pediatric patients under Japanese medical practice

This is a US multi-center, prospective, real world, observational drug registry enrolling patients actively treated with Uptravi. Participating patients will be followed prospectively for a maximum of 18 months from the date of enrollment into the registry.

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

This was a multicenter, prospective, observational, open-label study. Patients meeting inclusion/exclusion criteria received treatment with treprostinil as recommended by their treating physicians and were followed according to standard of care. This observational study collected clinical data and biologic specimens from patients who were treated for portopulmonary hypertension (PoPH), with a goal of achieving hemodynamic parameters appropriate for orthotopic liver transplantation candidacy, including mean pulmonary arterial pressure (mPAP) less than 35 mmHg and pulmonary vascular resistance (PVR) less than 3 Wood-units (WU) at Week 24 in patients with severe PoPH.

The purpose of this study is to provide sildenafil therapy to eligible adult patients with pulmonary arterial hypertension (PAH) and to the patients who completed the A1481142 study for the treatment of PAH in Poland. Only safety and tolerability data will be collected in these patients.

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.

Most patients with mitral valve disease are symptomatic with shortness of breath and a limited activity level prior to mitral valve surgery. Despite surgical repair or replacement of the mitral valve, many patients remain symptomatic with an impaired ability to live an active lifestyle. Often after extensive evaluation, no other pulmonary, left ventricular dysfunction, or valvular heart disease is responsible for the continued symptoms, and some of these patients will be limited by persistent pulmonary hypertension (PH) at rest or with exertion that is responsible for limiting their activity level and impacting their quality of their life. It is our goal in the proposed study to systematically characterize symptomatic and asymptomatic patients greater than six months after mitral valve surgery using clinical data, echocardiographic evaluation, laboratory assessment, and in some patients, invasive hemodynamic measurements. The investigators will screen asymptomatic and symptomatic patients with resting echocardiography and also with echocardiography during exercise, as many patients will exhibit exercise-induced PH following mitral valve surgery. Pulmonary artery (PA) pressure will be estimated from echocardiography using Doppler-derived calculations. If elevated PA pressures are observed with echocardiography, then symptomatic patients will undergo right heart catheterization for invasive pressure measurement, which is the gold-standard for the diagnosis of PH. When PH is present and there is a normal wedge pressure (PCWP) during invasive pressure measurement, further assessment to identify potential candidates for PH therapy will be performed. This involves having patients breathe inhaled nitric oxide, a rapid-acting, pulmonary vasodilator with a short half-life. While breathing inhaled nitric oxide, blood pressure, PA pressure, PCWP, and cardiac output will be monitored to characterize individuals who could benefit symptomatically from pharmacotherapy to treat underlying PH. It is important to note that only a small minority of patients exhibit a positive vasodilator response and those with PH and a normal PCWP without an initial vasodilator response would still be identified as candidates for chronic PH therapy. The information generated from this proposed research will make a significant contribution to the understanding of PH in a group of patients in whom it has not been previously studied. Scientific reports on the evaluation of patients with PH after mitral valve surgery are almost nonexistent from the modern era. Furthermore, patients with PH due to mitral valve disease have been excluded from clinical trials of agents currently approved by the U.S. Food and Drug Administration (FDA) to treat PH. Therefore, this work will carefully characterize PA pressures in an objective manner in a group of patients following mitral valve surgery who remain limited with respect to their activity levels. In addition, the investigators will gain a better understanding of the frequency with which patients have PH and a normal PCWP, which identifies a cohort of patients who could have an improvement in their symptoms and quality of life with chronic vasodilator treatment.

Understudied drugs will be administered to children per standard of care as prescribed by their treating caregiver and only biological sample collection during the time of drug administration will be involved. A total of approximately 7000 children aged <21 years who are receiving these drugs for standard of care will be enrolled and will be followed for up a maximum of 90 days. The goal of this study is to characterize the pharmacokinetics of understudied drugs for which specific dosing recommendations and safety data are lacking. The prescribing of drugs to children will not be part of this protocol. Taking advantage of procedures done as part of routine medical care (i.e. blood draws) this study will serve as a tool to better understand drug exposure in children receiving these drugs per standard of care. The data collected through this initiative will also provide valuable pharmacokinetic and dosing information of drugs in different pediatric age groups as well as special pediatric populations (i.e. obese).

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