Active clinical trials for "Familial Primary Pulmonary Hypertension"

This study proposes the use of a mobile health intervention (utilizing a smart phone app) to encourage increased exercise in PAH patients. The study will be a randomized trial to examine feasibility of an mHealth (mobile device) Fitbit Charge HR and cell phone application intervention to improve step counts and increase participants activity level as compared to no intervention. The Fitbit Charge Heart Rate (HR) monitors activity and the cell phone application provides encouragement notifications to half the subjects while the other half do not receive encouragements.

The main purpose of this clinical trial is to examine the feasibility and effects of tamoxifen in subjects with pulmonary arterial hypertension (PAH). The study will evaluate how well the drug is tolerated, and its impact on functional condition and selected biomarkers. Changes in tricuspid annular plane systolic excursion (TAPSE) and other parameters determined by transthoracic echocardiography will be evaluated as well as changes in additional metrics such as six minute walk test distance, quality of life assessments, and hormone levels.

This is an open-label study for patients who participated in the BPS-MR-PAH-203 study and have volunteered to continue treatment for PAH with Beraprost Sodium Modified Release (BPS-MR) tablets.

This is a 24-month, multi-center, prospective, open-label, safety evaluation in PAH subjects following transition from a stable dose of inhaled iloprost (Ventavis). Subjects are to be evaluated for safety throughout the course of the study while secondary assessments will be conducted at Baseline, Week 6, Week 12, and Months 6, 12, 18 and 24 following initiation of treprostinil sodium.

Our hypothesis is that IV or SQ Treprostinil can improve 6 minute walk distance, hemodynamics and quality of life in patients with interstitial lung disease and severe secondary pulmonary arterial hypertension.

The main objective of the AC 055 303/SERAPHIN OL study, which will follow the AC 055 302/SERAPHIN study, will be to assess the long-term safety and tolerability of ACT 064992 in patients with symptomatic PAH.

Patients with symptomatic idiopathic (IPAH) or familial (FPAH) pulmonary arterial hypertension in New York Heart Association (NYHA) class II to IV , naive to PAH treatment or currently being treated with a stable dose of either bosentan or sildenafil and who complete PROWESS 15 will be enrolled in the PROWESS 15 Extension study. This is a double-blind (12 week), randomized study to compare the safety and tolerability of inhaled iloprost power disc-15 and power disc-6 in patients with symptomatic pulmonary arterial hypertension (PAH). After completion of the double blind period, patients will be entered in the open label period using iloprost power disc-15.

The purpose of this study is to determine whether the addition of sapropterin dihydrochloride (6R-BH4) to existing treatment has any effect in patients with pulmonary arterial hypertension (PAH). Patients with PAH have low levels of a substance called nitric oxide (NO). Tetrahydrobiopterin (BH4) is a substance produced by the body that is an essential requirement in the formation of NO. NO is thought to be helpful in keeping blood vessels in the lung healthy. 6R-BH4 is an experimental (unproven) medicine made in the lab that is very much like the BH4 that our own body makes. The researchers are investigating whether 6R-BH4 can be added safely to current treatment for PAH and whether there is any evidence of benefit from its use. The study will take approximately one year to complete from the time recruitment begins. The primary objective of the study is to evaluate the safety of oral 6R-BH4, administered in escalating doses in addition to standard care, in subjects with pulmonary arterial hypertension (PAH). The secondary objective of the study is to evaluate change in biochemical markers of endothelial dysfunction and nitric oxide synthetase activity (coupled and uncoupled) in subjects with PAH receiving escalating doses of oral 6R-BH4 in addition to standard care. The third objective of the study is to evaluate change in biomarkers of disease progression, 6-minute walk (6MW) distance, Borg dyspnea scores, and quality of life (QOL) measures in subjects with PAH receiving escalating doses of oral 6R-BH4 in addition to standard care.

The purpose of this study is to assess the safety and tolerability of sorafenib in patients with PAH already on existing therapy with a prostacyclin [epoprostenol (Flolan)], treprostinil (Remodulin), or iloprost alone, or with or without sildenafil (Viagra/Revatio).

This study evaluates the effects of bosentan on oxygen saturation, hemodynamics and exercise capacity in patients with pulmonary arterial hypertension related to Eisenmenger physiology. Patients receive bosentan or placebo for 16 weeks.