Contribution of Echocardiography to Prognostic Evaluation of Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionThe purpose of this study is to determine the prognostic value of echocardiographic parameters in comparison with clinical and hemodynamic parameters in pulmonary arterial hypertension (PAH). A secondary purpose of this study is to analyze the disease evolution after 3 to 6 months. In pulmonary fibrosis it has been demonstrated that the variation of clinical and paraclinical parameters between 2 examinations has a prognostic interest. In this study the prognostic value of variation of some echocardiographic parameters between initial examination and echocardiography after 3 or 6 months will be evaluated. Another secondary purpose is to create a common database for Pneumology, Cardiology and Epidemiology departments with prospective registration of new cases of PAH and follow of patients under treatment.
Qualitative Research to Explore Treatment Preference in Pulmonary Arterial Hypertension (PAH) Subjects...
HypertensionPulmonaryGlaxoSmithKline (GSK) is embarking on a clinical program to assess the treatment of PAH with an Angiotensin converting enzyme 2 (ACE2). This new treatment may require subcutaneous administration, in comparison to current treatments which are taken orally. Hence, GSK would like to conduct this qualitative interview study with PAH subjects to explore subject's perspective and preferences for various modes of treatment administration (daily or weekly subcutaneous injection versus current treatment options). This will be a qualitative study comprising the conduct of semi-structured telephone concept elicitation interviews with 8 to 10 PAH subjects (each approximately 30 minutes in duration) in the United States (US).
Pulmonary Artery Involvement in Takayasu's Arteritis
Takayasu ArteritisPulmonary Artery HypertensionThe purpose of this study is to analyze the clinical manifestations, imaging features, and prognosis-related factors of pulmonary artery (PA) involvement in Takayasu's arteritis (TA), and to explore the early clinical features of PA involvement in TA patients.
Prevalence of the Hyperventilation Syndrome in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionDyspnea is a major symptom in pulmonary arterial hypertension and people with the same haemodynamic have generally different degree of dyspnea in pulmonary arterial hypertension. The hyperventilation syndrome is a frequent cause of dyspnea in general population and in respiratory diseases like asthma but has never been studied in pulmonary hypertension. The goal of this study is to measure the prevalence of pulmonary hypertension in a population of patients with controlled pulmonary arterial hypertension (PAH).
Prevalence of Pulmonary Hypertension (PAH) in Patients With Thalassemia
Thalassemia MajorThalassemia Intermedia1 moreThis is a multicenter observational case-control analysis lasting 12 months aimed at determining the prevalence of pulmonary hypertension (PAH) in patients with Thalassemia Major and Intermedia. The patients will be followed, treated and examined according to the best standard clinical practice dictated by the Italian Society for the study of Hemoglobinopathies (SITE), Thalassemia International Federation (TIF)and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of European Society of Cardiology (ESC); European Respiratory Society (ERS); International Society of Heart and Lung Transplantation (ISHLT) guidelines.
Pulmonary Arterial Hypertension in Patients With Direct-Acting Antiviral Medications for Hepatitis...
Pulmonary Artery HypertensionChronic Hepatitis cThe purpose of this study is the evaluation of PAH in patients, who are treated with DAA medication for HCV infection.
A Systematic Review of Factors Associated With Pulmonary Arterial Hypertension in Systemic Sclerosis...
Pulmonary Arterial HypertensionSystemic SclerosisThis protocol is of a systematic review for risk factors of pulmonary arterial hypertension in systemic sclerosis.
4DFlow Magnetic Resonance Imaging in Patients With Pulmonary Hypertension Associated With Congenital...
Congenital Heart DiseasePulmonary Arterial HypertensionCongenital heart disease is the most common congenital anomaly. The life expectancy of children with congenital heart disease has increased considerably in recent years. Nevertheless, the evolution of these patients is marked by an increased risk of complications. Arrhythmias, heart failure, pulmonary arterial hypertension (PAH) and endocarditis may be promoted by the absence or delay of management in childhood, by residual lesions or post-operative cardiac scars and by the presence of prosthetic materials. PAH is a common complication of congenital heart disease, especially in non-operated shunts. PAH corresponds to an increase in pulmonary vascular resistance and mean pulmonary arterial pressure that becomes greater than 25mmHg at rest, leading to right ventricular failure and ultimately to the patient's death. Eisenmenger's syndrome corresponds to a non-reversible pulmonary arterial hypertension with a left-right shunt initially left open, then right-left secondary to the increase in pulmonary vascular resistance, leading to cyanosis, polycythemia and multivisceral involvement. It is the most advanced form of PAH with congenital heart disease. PAH will be suspected during echocardiographic follow-up of any patient with congenital heart disease, on the analysis of the velocity of tricuspid and/or pulmonary regurgitation flow. Echocardiography allows the monitoring of the VD (right ventricle) function, which is the major prognostic element in PAH. Cardiac catheterization is systematically recommended and remains the gold standard to confirm the diagnosis of PAH, establish its pathophysiology and prognosis but also for the follow-up under medical treatment of these patients in tertiary centres every 6 months. Although this tool is the gold standard, rigorously performed, it remains an invasive examination often poorly experienced by patients. 4D Flow MRI is a promising imaging that allows the acquisition of anatomical, volume, right ventricular remodeling and intracardiac flow information in a single step with 2D (only 8 minutes extra), in free breathing and totally autonomous mode. Thus, at the same time as the realization of a 2D MRI, essential for the diagnosis and follow-up of PAH, with an additional 8 minutes for 4D flow, the investigators could have additional fundamental information on pulmonary cardiac output but also prognostic markers of right ventricular dysfunction turning dramatic in pulmonary vascular disease.
Insulin- and Glucose Metabolism in Pulmonary Arterial Hypertension
Insulin ResistanceInsulin resistance is a key mechanism in metabolic disorders, which has also been implicated in the development of pulmonary hypertension. In this pilot study, the investigators´ goal is to directly determine insulin sensitivity in idiopathic pulmonary arterial hypertension patients and to compare the results with data from healthy controls.
Dysregulation of Lipid Metabolism and Right Ventricular Function in PAH
Pulmonary Arterial HypertensionRight ventricular (RV) failure is the predominant cause of death in pulmonary arterial hypertension (PAH). No RV-specific therapies are available, in part because the underlying mechanisms of RV dysfunction are poorly understood. Given the heart's preference for fatty acids (FA) as an energy source, a deeper understanding of FA metabolism may shed light on RV adaptation to elevated afterload in PAH. The purpose of this study is to test the hypothesis that defects in fatty acid metabolism are common in PAH and contribute to RV failure. The investigators will measure peripheral and transcardiac lipid and glucose metabolites in PAH patients in comparison with patients with pulmonary venous hypertension and no evidence of pulmonary hypertension. The investigators will also correlate metabolites with concurrent measurement of right ventricular function.