Atrial Septal Defect - Exercise Capacity and Pulmonary Hypertension
Atrial Septal DefectPulmonary HypertensionThe purpose of the study is to compare exercise capacity, cardiac contractility, pulmonary vascular pressures and heart rate variability between patients with an atrial septal defect and healthy controls.
CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH
Interstitial Lung DiseaseScleroderma1 morePatients with interstitial lung disease (ILD) and scleroderma who develop pulmonary hypertension (PH) do not fit well into the current classification system and treatments for pulmonary hypertension. This study aims to better understand patients with ILD-PH and scleroderma and to determine if treatment with Macitentan is beneficial.
The Role of Transthoracic Echocardiography in Diagnosis of Pulmonary Hypertension of Infants With...
Pulmonary HypertensionBronchopulmonary DysplasiaPulmonary hypertension may develop in premature newborn infants due to impaired lung development. The diagnosis of this disease can actually be made with interventional methods. In this study, we evaluated the importance of echocardiographic examination and blood laboratory tests in diagnosing this disease.
Network Medicine Approaches to Classify Heart Failure With PReserved Ejection Fraction by Signatures...
Heart FailureDiastolic4 moreHeart failure (HF) is a syndrome, resulting from structural or functional impairment of ventricular filling or ejection of blood. Effective HF management depends on accurate and rapid diagnosis requiring assessment of symptoms and physical signs in combination with advanced and expensive imaging tools. However, several challenges arise from the traditional symptom-based diagnosis because co-morbidities of HF have similar presentations. This implies the need for a deeper knowledge of mechanistic links among genetic and epigenetic events governing the pathophysiology of HF leading to a novel molecular-based system to differentiate HF phenotypes. Now, it is emerging that the pathophysiology of HFpEF and HFrEF is different, it provides an opportunity to identify biomarker candidates that could aid in HF diagnosis and stratification between these two forms of the disease. The aim of PRESMET project is to perform liquid biopsy strategies to identify novel putative non-invasive epigenetic-sensitive biomarkers that could be used either alone or in combination with established diagnostic tests, such as natriuretic peptide, to help differentiate HFpEF from HFrEF. The Investigators will perform DNA methylation analysis on CD4+ T cells isolated from patients versus controls. Remarkably, big data generated from NGS tools will be analyzed by advanced network-oriented algorithms. Our results may provide a useful clinical roadmap in order to improve precision medicine and personalized therapy of HF.
A Study to Assess the Safety and Efficacy of Treprostinil to Facilitate Liver Transplantation in...
Portopulmonary HypertensionPulmonary Arterial Hypertension1 moreThis was a multicenter, prospective, observational, open-label study. Patients meeting inclusion/exclusion criteria received treatment with treprostinil as recommended by their treating physicians and were followed according to standard of care. This observational study collected clinical data and biologic specimens from patients who were treated for portopulmonary hypertension (PoPH), with a goal of achieving hemodynamic parameters appropriate for orthotopic liver transplantation candidacy, including mean pulmonary arterial pressure (mPAP) less than 35 mmHg and pulmonary vascular resistance (PVR) less than 3 Wood-units (WU) at Week 24 in patients with severe PoPH.
To Provide Sildenafil Therapy To Eligible Adult Patients With Pulmonary Arterial Hypertension For...
Pulmonary HypertensionThe purpose of this study is to provide sildenafil therapy to eligible adult patients with pulmonary arterial hypertension (PAH) and to the patients who completed the A1481142 study for the treatment of PAH in Poland. Only safety and tolerability data will be collected in these patients.
Symptomatic Pulmonary Arterial Hypertension After Mitral Valve Surgery
Pulmonary HypertensionMitral Valve DiseaseMost patients with mitral valve disease are symptomatic with shortness of breath and a limited activity level prior to mitral valve surgery. Despite surgical repair or replacement of the mitral valve, many patients remain symptomatic with an impaired ability to live an active lifestyle. Often after extensive evaluation, no other pulmonary, left ventricular dysfunction, or valvular heart disease is responsible for the continued symptoms, and some of these patients will be limited by persistent pulmonary hypertension (PH) at rest or with exertion that is responsible for limiting their activity level and impacting their quality of their life. It is our goal in the proposed study to systematically characterize symptomatic and asymptomatic patients greater than six months after mitral valve surgery using clinical data, echocardiographic evaluation, laboratory assessment, and in some patients, invasive hemodynamic measurements. The investigators will screen asymptomatic and symptomatic patients with resting echocardiography and also with echocardiography during exercise, as many patients will exhibit exercise-induced PH following mitral valve surgery. Pulmonary artery (PA) pressure will be estimated from echocardiography using Doppler-derived calculations. If elevated PA pressures are observed with echocardiography, then symptomatic patients will undergo right heart catheterization for invasive pressure measurement, which is the gold-standard for the diagnosis of PH. When PH is present and there is a normal wedge pressure (PCWP) during invasive pressure measurement, further assessment to identify potential candidates for PH therapy will be performed. This involves having patients breathe inhaled nitric oxide, a rapid-acting, pulmonary vasodilator with a short half-life. While breathing inhaled nitric oxide, blood pressure, PA pressure, PCWP, and cardiac output will be monitored to characterize individuals who could benefit symptomatically from pharmacotherapy to treat underlying PH. It is important to note that only a small minority of patients exhibit a positive vasodilator response and those with PH and a normal PCWP without an initial vasodilator response would still be identified as candidates for chronic PH therapy. The information generated from this proposed research will make a significant contribution to the understanding of PH in a group of patients in whom it has not been previously studied. Scientific reports on the evaluation of patients with PH after mitral valve surgery are almost nonexistent from the modern era. Furthermore, patients with PH due to mitral valve disease have been excluded from clinical trials of agents currently approved by the U.S. Food and Drug Administration (FDA) to treat PH. Therefore, this work will carefully characterize PA pressures in an objective manner in a group of patients following mitral valve surgery who remain limited with respect to their activity levels. In addition, the investigators will gain a better understanding of the frequency with which patients have PH and a normal PCWP, which identifies a cohort of patients who could have an improvement in their symptoms and quality of life with chronic vasodilator treatment.
Inhaled Iloprost in the Treatment of Patients With Pulmonary Hypertension up to 4 Years
Pulmonary HypertensionThis is an open-label, uncontrolled, prospective long-term observation of Specific Drug in the treatment of patients with pulmonary hypertension up to 4 years. 160 patients with primary (idiopathic and familial) pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) due to scleroderma with New York Heart Association (NYHA) functional class and exercise capacity, defined as class III and IV planned to enroll. Efficacy, safety and tolerability of the drug and the survival of the patients will be observed.
Genetic and Environmental Determinants That Control Metabolism in Pulmonary Hypertension
Iron-sulfur Cluster DeficiencyPulmonary Hypertension1 moreThe investigators are performing this research study to determine whether having low iron-sulfur cluster levels can cause a disease known as pulmonary hypertension (PH). PH is defined as abnormally high blood pressure in the arteries of the lungs. Usually, small specialized structures inside each human cell called mitochondria are in charge of generating energy within lung arteries for normal function. During situations of disease or stress, lung arteries undergo a change in the function of mitochondria, resulting in the development of PH. In studies on mice, investigators have learned that alterations in the production of specific metal complexes called iron-sulfur clusters are responsible for these changes. This makes it more likely that mice will develop PH. In this study, the investigators want to find out if alteration of iron-sulfur cluster formation leads to increased likelihood of developing PH in humans.
Non-interventional Study on Compliance of Inhaled Treatment With Ventavis in Patient With Pulmonary...
HypertensionPulmonaryLocal, prospective, multicenter, non-comparative, non-interventional, observational study. It is planed to assess compliance of patients with PH (pulmonary hypertension) with the physician's recommendation in real practice.