Safety and Efficacy Study of Bosentan in Progressive Pulmonary Sarcoidosis
SarcoidosisPulmonary HypertensionProgressive pulmonary sarcoidosis occurs in up to twenty percent of patients who require persistent treatment, but available treatment options have shown considerable long-term toxicity and uncertain or unproven efficacy. In these patients, pulmonary fibrosis and pulmonary hypertension are common complications which have major prognostic impact. Endothelin-1 (ET-1) has been demonstrated to play a key role in pulmonary fibrosis and pulmonary hypertension, and a potential role in pulmonary sarcoidosis. ET-1 is a potent vasoconstrictor and can promote fibrosis, cell proliferation, and remodeling, and is pro-inflammatory. Preliminary data have shown the therapeutic potential of the endothelin receptor antagonist (ERA) bosentan in sarcoidosis associated pulmonary hypertension. In this light, the therapeutic potential of bosentan as an add-on treatment in progressive pulmonary sarcoidosis needs to be evaluated.
Pharmacokinetic Study of Milrinone in Babies With Persistent Pulmonary Hypertension of the Newborn...
Persistent Fetal Circulation SyndromeThe purpose of this pilot study is to determine a safe dose of milrinone to use in a larger study of babies with persistent pulmonary hypertension of the newborn (PPHN).
Hydroxyurea for Children and Young Adults With Sickle Cell Disease and Pulmonary Hypertension
Sickle Cell DiseasePulmonary HypertensionThe goal of this study is to test the hypothesis that hydroxyurea is effective for the specific treatment of secondary pulmonary hypertension found on screening in children and young adults with sickle cell disease.
Chronic Sildenafil for Severe Diaphragmatic Hernia
HerniaDiaphragmatic4 moreThe purpose of this study is to test if sildenafil is effective in the treatment of infants with severe congenital diaphragmatic hernia (determined by the presence of prolonged pulmonary hypertension or prolonged oxygen supplementation on mechanical ventilation), as measured by the estimated pulmonary artery systolic pressure following treatment.
Dimethyl Fumarate (DMF) in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension
Systemic SclerosisPulmonary; HypertensionA double-blinded, placebo-controlled study of Dimethyl fumarate (DMF) in 34 Systemic Sclerosis-Pulmonary Hypertension (SSc-PAH) patients. The study will determine safety and the primary outcome variability for DMF in treating SSc-PAH; the primary outcome of clinical efficacy in this pilot trial will be improvement in 6-minute walk distance (6MWD).
Extended Access Program to Assess Long-term Safety of Bardoxolone Methyl in Patients With Pulmonary...
Pulmonary HypertensionThis extended access study will assess the long-term safety and tolerability of bardoxolone methyl in qualified patients with pulmonary hypertension (PH) who previously participated in controlled clinical studies with bardoxolone methyl.
A Phase 3 Adaptive Study to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Participants...
Pulmonary HypertensionChronic Obstructive Pulmonary DiseaseThe primary objective of this study is to demonstrate the efficacy of inhaled treprostinil compared to placebo in improving exercise ability as measured by change from baseline in 6-Minute Walk Distance (6MWD) following 12 weeks of active treatment in participants with PH-COPD.
An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE...
Pulmonary HypertensionInterstitial Lung Disease1 moreThis is a multicenter, open-label trial to evaluate the safety and efficacy of inhaled treprostinil in subjects with pre-capillary pulmonary hypertension (PH) associated with interstitial lung disease (ILD) including combined pulmonary fibrosis and emphysema (CPFE). The study will include about 266 patients who completed all required assessments in the RIN-PH-201 study at approximately 100 clinical trial centers. The study will continue Your participation in this study is voluntary and will last until you discontinue from the study or the study ends. The study will continue until each subject reaches the Week 108 visit or until inhaled treprostinil become commercially available for patients with PH associated with ILD including CPFE (whichever is sooner).
A Research Study To Assess The Effectiveness And Safety Of Different Doses Of Oral PF-00489791 In...
HypertensionPulmonaryStudy will assess PF-00489791 efficacy and safety in Pulmonary Arterial Hypertension (PAH)
Study Providing Monotherapy (Sitaxsentan) And Combination Therapy (Sitaxsentan+Sildenafil) To Subjects...
Pulmonary Arterial HypertensionPulmonary HypertensionAs sitaxsentan is the agent most highly selective for ETA (Endothelin Type A (receptor)), and does not significantly impact sildenafil pharmacokinetics the combination of most promise for pulmonary arterial hypertension (PAH) therapy is these two oral drugs administered in combination.