Evaluation of the Strength of Handgrip in Patients With Pulmonary Artery Hypertension
Pulmonary HypertensionOur study aims to investigate the changes in muscle strength and muscle mass in PAH patients compared to healthy individuals and determine its effects on prognosis. Further categorization of PAH patients based on their NYHA class will help determine if their handgrip strength decreases while progressing from NYHA class 1 to 4. This in turn allows us to examine if the handgrip test can be considered as an alternative to a 6-minute walking test (6MWT). The Jamar Hydraulic Hand Dynamometer device in our hospital is used for measuring the handgrip strength test. The data of the included subjects in this study are obtained and recorded from the existing files. Also, the handgrip strength test data will be recorded after the investigation
Natural History Study of Biomarkers in Pulmonary Arterial Hypertension
Pulmonary DiseasePulmonary HypertensionBackground: - High blood pressure in the lungs, known as pulmonary arterial hypertension (PAH), is a rare disorder. Some people have disease-associated PAH and some have PAH from an unknown cause. Researchers want to follow the natural history of all PAH patients to understand how PAH progresses in order to discover targets for future research into new treatments. To further identify treatment targets, they will compare healthy volunteers to patients with PAH. Objectives: - To study the natural history of PAH. Eligibility: Individuals at least 18 years of age who have PAH. Healthy volunteers at least 18 years of age. Design: Participants with PAH will have periodic visits to the National Institutes of Health Clinical Center. After the first visit, they will return in 6 months and then yearly or every other year for as long as the study continues. The first visit will take up to 3 days. It will involve the following tests: Physical exam and medical history Blood and urine samples Heart and lung function tests and imaging studies Six-minute walk test Questions about exercise and physical activity Healthy volunteers will have only one visit to the Clinical Center, during which they will undergo screening tests, and complete many of the same tests as patients with PAH
Hormonal, Metabolic, and Signaling Interactions in PAH
Idiopathic Pulmonary Arterial HypertensionHeritable Pulmonary Arterial Hypertension2 moreOur hypothesis is that optimal treatment of the dysfunctional metabolic pathways which underlie PAH will improve pulmonary vascular function and consequences of the disease.
TROPHY PAH Pivotal Study - TReatment of Pulmonary HYpertension for PAH Pivotal Study
Pulmonary Arterial HypertensionTheis is a prospective, multicenter, blinded, randomized sham controlled pivotal clinical trial with a crossover at 6M, to assess the safety and effectiveness of pulmonary artery denervation with the TIVUS™ System in subjects with PAH. The study will assess improved and/or maintained exercise tolerance in patients with PAH through the analysis of exercise tolerance, hemodynamic changes, clinical worsening and the quality of life who got treated by the TIVUS system.
Pharmacokinetics, Pharmacodynamics, and Safety Profile of Understudied Drugs Administered to Children...
Coronavirus Infection (COVID-19)Pulmonary Arterial Hypertension23 moreThe study investigators are interested in learning more about how drugs, that are given to children by their health care provider, act in the bodies of children and young adults in hopes to find the most safe and effective dose for children. The primary objective of this study is to evaluate the PK of understudied drugs currently being administered to children per SOC as prescribed by their treating provider.
Medication Reconciliation in Pulmonary Hypertension
Pulmonary HypertensionPulmonary Hypertension Chronic Thromboembolic1 morePulmonary hypertension (PH) is a life threatening condition. In PH, pulmonary arterial hypertension (PAH) and chronic thrombo-embolic chronic pulmonary hypertension (CTEPH) are two rare diseases requiring specific and complex drug management. In France ,a part of these treatments ,only available in hospital pharmacies, are generally unknown from community health care professionals despite the high risk of drug-interactions and side effects. Anticipating medication errors at the begging of the disease is therefore important, and could be done through medication reconciliation.
HRV as a Marker of Treatment Response in PAH Arterial Hypertension
Pulmonary Arterial HypertensionThis is a prospective longitudinal cohort study to determine the value of HRV obtained using noninvasive actigraphy to quantify the response to pulmonary vasodilator therapy in newly diagnosed PAH patients
Exercise Hemodynamic, Right Ventricular Coupling and Echocardiography in Pulmonary Hypertension...
HypertensionPulmonary2 moreThis study aims to investigate the exercise profile in pulmonary hypertension patients with either pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension or pulmonary hypertension due to left heart disease and in disease control.
Assessment of the TGF-beta Pathway and Micro-RNA in Pediatric Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionThis is a prospective pilot study to assess the plasma levels of particular proteins involved in the transforming growth factor beta (TGF-β) pathway and its down stream regulators, CHIP, as well as micro RNA molecules in subjects with pulmonary arterial hypertension (PAH) and compare them to control subjects without PAH to see if they can be used as a diagnostic or prognostic marker of PAH and how this compares to other diagnostic biomarkers N-terminal pro-natriuretic peptide (NT Pro-BNP) and C-reactive protein (CRP).
Fetal Endoscopic Tracheal Occlusion for Congenital Diaphragmatic Hernia (FETO)
Congenital Diaphragmatic HerniaPulmonary Hypoplasia1 moreThe purpose of this research is to gather information on the safety and effectiveness of a new procedure called Fetoscopic Endoluminal Tracheal Occlusion (FETO).