Active clinical trials for "Familial Primary Pulmonary Hypertension"

The investigators planned a randomized controlled study to investigate the effects of adding yoga respiratory training to osteopathic manipulative treatment (OMT), and OMT alone on exhaled nitric oxide level and cardiopulmonary function in patients with pulmonary arterial hypertension (PAH). Our hypothesis is that combined intervention including OMT and yoga respiratory training may improve exhaled nitric oxide level and cardiopulmonary function in patients with PAH.

The objectives of this study are to evaluate the efficacy and safety of sotatercept (MK-7962) treatment (plus background pulmonary arterial hypertension (PAH) therapy) versus placebo (plus background PAH therapy) at 24 weeks in adults with PAH. The primary hypothesis of the study is that the participants receiving sotatercept will have improved 6-minute walk distance (6MWD) at 24 weeks compared to participants receiving placebo.

The development of selexipag for intravenous administration will be useful to avoid treatment interruptions in patients with pulmonary arterial hypertension (PAH) already treated with selexipag administered orally as tablets (Uptravi®). The target population for intravenous selexipag includes those PAH patients who are hospitalized and are unable to swallow tablets of Uptravi. The primary objective of this study is to assess whether it is safe for patients with PAH to temporarily change from selexipag tablets (Uptravi®) to selexipag given directly into a vein (intravenous selexipag), and then switching back to the initial oral dose of selexipag.

This was an open-label extension of Study RIV-PH-402, TRUST-1: Treprostinil for Untreated Symptomatic Pulmonary Arterial Hypertension (PAH) Trial. Subjects who completed Study RIV-PH-402 were eligible to enroll.

Pulmonary arterial hypertension (PAH) can result in right ventricular failure and death. Anakinra has been used in patients with left sided heart failure, and the present study looks to determine if anakinra is safe and effective in patients with PAH. To accomplish this goal, we plan to evaluate for exercise improvement (as assessed by cardiopulmonary exercise testing) in 10 patients with PAH on anakinra.

The purpose of the study is to evaluate the safety and tolerability of a rapid dose titration regimen of subcutaneous Remodulin® therapy in patients with PAH.

The main purpose of this clinical trial is to examine the feasibility and effects of fulvestrant in post-menopausal women with pulmonary arterial hypertension (PAH). The study will evaluate how well the drug is tolerated. The study will evaluate changes in circulating hematopoietic progenitor cells, plasma hormone levels, NT-proBNP, and other plasma biomarkers after the administration of fulvestrant. Changes in tricuspid annular plane systolic excursion, stroke volume index, right ventricular fractional area change, and other echo parameters after fulvestrant administration will be evaluated as well as changes in distance walked in six minutes.

Rationale: Nutrition and lifestyle interventions are currently not implemented in usual clinical care of PAH-patients. Mainly because there is little known on the relation between pathology, nutrition and lifestyle. Patients who suffer from Pulmonary Arterial Hypertension feel insecure about their nutrition and lifestyle. The investigators hypothesize that an intervention on nutrition and lifestyle can improve the patients' quality of life. Objective: To explore the effect of a nutrition and lifestyle intervention on quality of life for patients suffering from PAH. Study design: Investigator initiated intervention study with control group. Study population: investigators aim to include 70 patients (18 - 80 years) with idiopathic, hereditable or drug related PAH, who have been stable for at least three months and are self-sufficient and/or have a family who's willing to participate in the lifestyle changes. Intervention (if applicable): Nutritional status, - education, - intervention and - compliance. Main study parameters/endpoints: This is an intervention study in which the investigators will asses the effect of a nutrition and lifestyle intervention on quality of life measured by SF-36 overall outcome with a significant difference of 6.35. Nature and extent of the burden and risks associated with participation, benefit and group relatedness: The burden for the patient exists of 12 extra visits to the hospital and contact moments, over a period of 11 months, as well compliance to the diet and lifestyle. There is minimal risk in participation.

This study aims to assess the safety and tolerability of the individual highest tolerated zamicastat doses, achieved in the study BIA-51058-201, during long-term treatment in Pulmonary Arterial Hypertension (PAH) disease.

An open label study to assess the safety and efficacy of tocilizumab in group 1 pulmonary arterial hypertension patients