Angiotensin Converting Enzyme (ACE2), Brain, Gut Dysbiosis in Pulmonary Hypertension
Pulmonary Arterial HypertensionPulmonary 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.
Pulmonary Hypertension in Interstitial Lung Disease
Pulmonary Arterial HypertensionDiffuse Interstitial Lung DiseaseHYPID study is an observational and prospective study of patients with interstitial lung disease and pre capillary hypertension diagnosed by right heart sided catheterization. The primary aim of the study is to identify prognostic factors.
Biomarkers in the Evaluation of Chronic Lung Disease
Pulmonary HypertensionPulmonary Arterial Hypertension1 moreWe hypothesize that hypoxia-induced pulmonary vascular remodeling is mediated by macrophage migration inhibitory factor (MIF), that remodeling is in fact the reflection of a chronic inflammatory process, and that MIF may be a useful biomarker of the severity and progression of both ILD and PH.
DNA Methylation Dynamics Underlying Arterial Remodeling in PAH Patients: CLEOPAHTRA Clinical Trial...
Pulmonary Arterial HypertensionPulmonary Arterial RemodelingTo identify epigenetic-sensitive modifications and novel biomarkers linked to pathogenesis of pulmonary arterial hypertension (PAH), we will perform the first study analyzing differentially-methylated regions (DMRs) in circulating T cells (CD04+ and CD08+) isolated from peripheral blood of patients undergoing right heart catheterization. Moreover, we will perform RNA deep sequencing on lung tissue biopsies to validate if DNA methylation signatures in circulating T cells could reflect perturbations of gene expression in lung tissues.
Evaluation of Respiratory Muscle in Patient With Pulmonary Arterial Hypertension
Pulmonary Artery HypertensionThe aim of the study is that evaluation of basic and accessory respiratory muscles and their relationship of six minute walk test in patient with pulmonary arterial hypertension (PAH)
Development of a New Tool for Dyspnea Measurement in Chronic Respiratory Diseases
COPD (With - Without Rehabilitation)Diffuse Interstitial Lung Diseases2 moreThe purpose of this study is the psychometric validation of a self-administered dyspnea questionnaire, usable in clinical practice in order to assess dyspnea and its impact on patients with chronic respiratory diseases.
Novel Screening Strategies for Scleroderma PAH
Pulmonary Arterial HypertensionSclerodermaPatients with scleroderma can develop heart failure due to high blood pressure in the lungs (a condition called pulmonary arterial hypertension). It is important to find pulmonary arterial hypertension early, so that it can be treated before heart failure develops. However, the tests that we now use to find the earliest form of this disease in scleroderma patients are not good enough. This study will examine whether tests performed during exercise can improve our ability to find early pulmonary arterial hypertension. The study will also try to identify genes that are responsible for the development of pulmonary arterial hypertension.
Screening for Sleep Hypoxemia in Pulmonary Arterial Hypertension
Sleep DisordersIn the investigators study, and regarding results of small cohorts in the literature, the investigators hypothesize that hypoxemia is frequent in IPAH and CPEPH. The investigators will explore these patients with a one night polysomnography and transcutaneous capnography, searching for hypoxemia and hypercapnia and by determining its physiopathologic mechanisms.
Tyvaso Dosing and Titration Evaluation: TyTRATE Registry
Pulmonary Arterial HypertensionThis prospective, observational, multi-center, patient registry will follow patients who are newly initiated on Tyvaso for the treatment of Pulmonary Arterial Hypertension (PAH). Once enrolled, the patients' dose and titration will be followed for the first 6 months of treatment with Tyvaso. A call-center will contact the patients directly at weeks 1, 2, 3, 4, 8, 12, 16, 20, and 24 to review their dose and titration schedule. In addition to patient-reported dosing data, some patient demographic information, will be collected by the investigative site at Baseline.
The Expression and Significance of MiRNA
Pulmonary Arterial HypertensionIt is not always known what causes PAH or what the best treatment is. The doctors doing this study would like to understand more about why some people develop PAH and other do not. They also would like to learn more about which treatments might help PAH and which people might respond better to treatments. Doctors think that testing certain substances found in blood cells might help answer these questions. These substances are normally released by our bodies to protect us from infection and to tell the difference between normal and foreign substances in our body. Finally, a new test will study very small molecules called microRNA that control how our genes are expressed. This study is being done to see if blood samples can be tested to determine who might develop PAH, how well drugs will work to treat PAH and to learn more about the development of PAH.