Acute Decompensation of Pulmonary Hypertension
The main objective of this study is to analyze the survival of a cohort of patients admitted for acute decompensation of pulmonary arterial hypertension or postembolic pulmonary hypertension and to establish the prognostic value of biomarkers.
ADAPT - A Patient Registry of the Real-world Use of Orenitram®
This prospective, observational, multicenter, patient registry will follow patients who are receiving treatment with Orenitram for the treatment of PAH for up to 78 weeks from Orenitram initiation
A Dose Escalation Study to Assess the Safety and Efficacy of Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis or Sarcoidosis
A phase 2b, open label study to assess the safety and efficacy of increasing doses of pulsed, inhaled nitric oxide (iNO) in subjects with pulmonary fibrosis and sarcoidosis on long term oxygen therapy followed by a long term extension study
ALlogeneic Cardiosphere-derived Stem Cells (CDCs) for Pulmonary Hypertension therApy
Pulmonary Arterial Hypertension or PAH is a progressive condition for which there is no cure. Even with substantial pharmacologic advances in the modern treatment era, survival still remains unacceptably poor, as reported in large PAH registries. Preclinical studies suggest that the administration of allogeneic CDCs have the potential to reduce adverse arteriolar remodeling in PAH which was the basis for the approved investigational new drug (IND). The use of CDCs as an adjunctive therapy in patients comprising 4 sub-groups of patients with PAH in which inflammation and immune dysfunction are key pathophysiologic drivers of PAH.
A Long-term Extension Study of Riociguat in Patients With Symptomatic Pulmonary Arterial Hypertension.
To provide riociguat therapy to eligible patients with PAH originating from Bayer-sponsored trials with BAY63-2521/ Riociguat / Adempas® who are currently or recently treated in these trials until lack of patient benefit as assessed by investigator, or commercial availability and reimbursement.
Ambrisentan in Patients With Porto-pulmonary Hypertension A Multicenter Open Label Trial
This is an Open Label, Multicenter, pilot clinical trial to assess the efficacy and safety of an oral selective Endothelin Receptor Antagonist (ambrisentan) in patients with portopulmonary hypertension. Preliminary evidence suggests that ambrisentan is safe and effective in patients with portopulmonary hypertension. The goal of therapy for these patients is to improve symptoms of dyspnea and to improve pulmonary hemodynamics to a mean pulmonary artery pressure <35 mm Hg in order to make patients eligible for liver transplantation. Therefore, the primary endpoints for this study will include 6 minute walk distance (6MWD) and pulmonary...
A Mobile Health Intervention in Pulmonary Arterial 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.
Analysis of the Severity and Prognosis of Pulmonary Hypertension Associated With Bronchiectasis.
Through analysis of demographic factors (gender, age, smoking history, body mass index), clinical indicators (signs and symptoms), sputum culture results (pseudomonas aeruginosa), Brain Natriuretic Peptide and imaging (scope and type of Bronchiectasis), pulmonary function index, inflammatory cytokines and inflammasome of patients, some factors will be found which maybe be related to severity and prognosis of Pulmonary Hypertension Associated With Bronchiectasis.
Angiotensin Converting Enzyme (ACE2), Brain, Gut Dysbiosis in Pulmonary Hypertension
Pulmonary arterial hypertension (PAH) is fatal with right heart failure due to raised pulmonary vascular pressure. Gut dysbiosis was identified in animals with pulmonary hypertension. Deidentified human samples will be tested for gut dysbiosis in PAH, circulating bacterial metabolites and markers of inflammation and gut leakiness. The gut microbiome and circulating metabolites, markers of inflammation and gut leakiness of PAH patients and healthy subjects will be compared in deidentified fecal samples and blood.
An Open Label Extension Study to Evaluate Inhaled Treprostinil in Adult PH With ILD Including CPFE
This 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...
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