Educational Training Influence in Patients With PAH(Pulmonary Artery Hypertension) on Inhaled Ventavis...
Pulmonary Arterial HypertensionVentavis it's a treatment for pulmonary hypertension, administered by an inhaler called I-Neb. In these patients, the non compliance of the treatment becomes its ineffective and increases the complications and mortality of the disease. It is therefore important to know which the adherence of patients to their treatment is and try to promote it. One possibility to reach a good level of adherence is the patient education regarding the drug administration and also to comply as better is possible the recommendations of the physician. For this reason, the objective of our study is to demonstrate improvement in the level of treatment compliance when they follow a strategy of continuing education, assessed by the device and software called Insight. The only special procedure to participate in the study then is that patients involved could receive more training than usual. Investigators expect involving in this study around 50 patients in treatment with Ventavis and diagnosed of Pulmonary Arterial Hypertension in 10-12 hospitals throughout Spain. The patients who participated in the study will be followed for 12 months from inclusion in the study and will perform around 5 visits in total.
Pulmonary Hypertension in Patients With Sickle Cell Disease in Nigeria
Secondary Pulmonary Arterial HypertensionSickle Cell Disease1 moreThis study will explore how people with sickle cell disease (SCD) develop a complication called pulmonary hypertension (PHTN), a serious disease in which blood pressure in the lungs is higher than normal. PHTN is also caused by HIV, hepatitis C and schistosomiasis. Patients who have both SCD and one of these other infections may develop more severe PHTN. The number of Nigerians with SCD who also have PHTN is not known, nor is the cause of PHTN in this population. This study will examine genetic material in people with and without SCD to determine whether certain genes will allow doctors to predict which patients with SCD are likely to develop PHTN. Nigerian males and females 10 years of age and older with or without SCD may be eligible for this study. Patients must have SS, SC, or SB thalassemia or other genotype; control subjects must have hemoglobin A or AS genotype. Participants undergo a complete medical history and physical examination, blood tests, electrocardiogram (EKG), ultrasound tests of the heart and abdomen, and a 6-minute walk (distance test) to determine exercise capacity. Blood tests include screening for HIV, hepatitis B and C, schistosomiasis, hookworm and malaria. Patients who test positive for HIV, hepatitis B or C, schistosomiasis, hookworm or malaria are referred for treatment at Ahmadu Bello University Teaching Hospital in Zaria, Nigeria, and those who test negative for hepatitis B are referred for vaccination. Genetic tests focus on genes involved in SCD, PHTN, inflammation, blood vessel function and red blood cell function.
Ventavis® Registry Protocol
Pulmonary Arterial HypertensionThe Ventavis® (iloprost) Registry is a multicenter, observational, U.S.-based study that longitudinally follows patients with pulmonary arterial hypertension (PAH) who have been receiving therapy with Ventavis® for at least 3 months. Patients diagnosed with WHO Group I PAH who are on a stable regimen of commercial Ventavis® will be followed for a maximum of 2 years from the time of enrollment. Data will be collected via patient interview and review of the medical record. Quarterly data collection will include capture of medications and Ventavis® adherence data.
Evaluating Pulmonary Arterial Hypertension in Patients With Systemic Sclerosis
Systemic SclerosisPulmonary HypertensionDoppler signals can be recorded from the lung parenchyma by means of a pulsed Doppler ultrasound system incorporating a special signal processing package- the Transthoracic Parametric Doppler (TPD) (Echosense Ltd., Haifa, Israel). Systemic sclerosis patients often develop pulmonary vascular disease leading to pulmonary hypertension. The TPD system may provide important insight into pulmonary blood vessels characteristics by the LDS (Lung Doppler Signals) signals that are related to pulmonary hypertension. The TPD performance in detecting PAH in SSc patients will be assessed in the study.
Cardiac Metabolic Remodeling After Pulmonary Vasodilator Therapy in Pulmonary Arterial Hypertension:...
Pulmonary Arterial HypertensionRight-Sided Heart FailurePulmonary arterial hypertension(PAH) is associated with the development of right heart failure. In the setting of heart failure, the heart shifts to increasing dependence on glucose metabolism. In this study, the investigators will perform cardiac positron emission tomography/magnetic resonance imaging (PET/MRI) scans to measure glucose metabolism in the heart before and after initiation of pulmonary vasodilator therapy for pulmonary arterial hypertension.
Impact of Inhaled Treprostinil Sodium on Ventilation Perfusion Matching
Pulmonary Arterial HypertensionChronic Obstructive Pulmonary DiseaseThe purpose of this study is to see how inhaled treprostinil sodium (Tyvaso) affects the amount of air and blood that reach the alveoli, or tiy air sacs, in the lungs of patients with Group 1 Pulmonary Arterial Hypertension with concomitant Chronic Obstructive Pulmonary Disease (COPD).
Early, Simple and Reliable Detection of Pulmonary Arterial Hypertension (PAH) in Systemic Sclerosis...
Systemic SclerosisPulmonary Arterial Hypertension1 moreA two-stage prospective observational cohort study in scleroderma patients to evaluate screening tests and the incidence of pulmonary arterial hypertension and pulmonary hypertension
Effects of Physical Training on Vascular Function as a Therapeutic Target in Pulmonary Hypertension...
Pulmonary Arterial HypertensionThe role of physical training in the treatment of pulmonary arterial hypertension (PAH) is controversial. The aim of the project is to evaluate the effect of physical training on markers of endothelial function and integrity and to identify those biomarkers associated with a better therapeutic response in patients with PAH and in an experimental model of pulmonary hypertension. Methodology: 1) Study in humans: sample size will be 50 patients with PAH. Responders and non-responders will be identified for a 12-week resistance training program. Before and after the physical training program, endothelial microparticles and circulating vascular progenitor cells, and metabolomic and mitochondrial function parameters in circulating endothelial cells will be analyzed. Patients will be identified in whom a more favorable response to the training program is obtained. Additionally, investigators will evaluate the relationship between this response and the biomarkers both at baseline and their change with the training program. 2) Study in a murine experimental model: investigators will study mice with pulmonary hypertension induced by the administration of Semaxanib (SU5416) and exposure to hypoxia for 3 weeks and control mice. Half of them will exercise on a treadmill for 3 weeks. At the end of the program the right ventricular pressure will be measured and the animals will be sacrificed. Morphometric studies in pulmonary and cardiac tissue, pulmonary endothelial function and metabolomic parameters in cardiac and skeletal muscle will be performed. Differences in these variables between the different experimental groups will be analyzed.
Determinants of Right Heart Remodeling in Patients With CTEPH or PAH
Chronic Thrombo-embolic Pulmonary Hypertension and Pulmonary Arterial HypertensionRight heart failure is the main cause of morbi-mortality in patients with pulmonary hypertension (PH), including patients of chronic thrombo-embolic pulmonary hypertension (CTEPH) or pulmonary arterial hypertension (PAH) etiologies. Endarterectomy is an effective treatment for patients with CTEPH to lower pulmonary pressure. Evidence of postoperative right heart remodeling are contrasted according to the studies and determinants of right heart failure are still unclear. Similarly, few evidence exists on right ventricular remodeling after bilateral lung transplantation for patients with pulmonary arterial hypertension (PAH). Recent evidence have supported the role of inflammation and immunity in the pathophysiology of PAH . While several cytokines have been shown to predict survival , little is known on the implication of inflammation and immunity in postoperative Right Ventricular failure in patients with PAH. • The specific translational goal of this current project is to elucidate the role of immune biomarkers in 6 months postoperative right heart adverse remodeling in patients with CTEPH or PAH. We speculate that selected immune biomarkers (such as CXCL9, interleukin -18 or interferon) and growth factors (such as HGF) are correlated with mid-term postoperative right heart failure. All consecutive adults with either CTEPH referred to our center for endarterectomy, or PAH referred for lung transplantation, will be included, aiming for 150 CTEPH and 50 PAH. After inclusion, patients will undergo assessment of right heart dimensions and function by cardiac magnetic resonance imaging (MRI, including 4-Dimensions blood flow sequences) and 2D and 3D trans-thoracic echocardiography (TTE), as well as immune panel analysis. All patients will undergo as part of routine care right heart catheterization within a week after TTE and MRI imaging. On the day of surgery, pulmonary pressure will be measured by right heart catheterization monitoring (as part of routine care) in order to estimate the drop of pressure and to adjust for the extent of endarterectomy for patients with CTEPH. TTE will also be performed on the day of surgery if possible. At 7 days post-endarterectomy or transplant, clinical outcomes will be collected and peripheral blood will be collected. Patients will be prospectively follow-up for 6 months. Death, need for reintervention, duration of vasopressor after surgery and number, duration and cause of readmission will be recorded. At 6 months after surgery, all survivors will undergo the same biological sampling, as well as an 4D MRI and a 2D and 3D TTE. Data of right heart catheterization at 6 months (as part of routine care) will be collected as well.
Exercise Capacity and Quality of Life in Patients With PPH Receiving Short Term Oral L-Citrulline...
Idiopathic Pulmonary Arterial HypertensionEisenmenger SyndromeDue to vasodilatory properties of the NO, one of the therapeutic approaches for IPAH is oral use of nitric oxide precursors (10). Efficacy of L-arginine is well-documented in the current literature but there is paucity of data with regard to L-citrulline- malate. Hence, this study will evaluate therapeutic efficacy of L-citrulline- malate in two categories of patients with pulmonary hypertension (IPAH, and Eisenmeger syndrome). This randomized clinical trial utilizes 6-minute walk, pro BNP levels and the echocardiographic indexes an indicator of functional improvement of the patients.