Selexipag for the Treatment of Schistosomiasis-Associated Pulmonary Arterial Hypertension
Pulmonary arterial hypertension (PAH) is a severe, progressive and potentially fatal disease that impairs the pulmonary circulation and leads to right ventricular failure. One of the world most prevalent etiologies of PAH is schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH). New drugs have emerged to treat other forms of PAH, but their benefits cannot be automatically translated for Sch-PAH patients, since this etiology was not included in the pivotal PAH trials. One of the most promising therapies for the treatment of PAH to emerge in recent years is selexipag, an oral IP receptor agonist, which acts on the ...
Side Effects of Pulmonary Hypertension Medications
The study seeks to analyze the patient reported effects of pulmonary hypertension medications and compare these with the side effects described on the package inserts. Side effects for these PH medications have been described in the adult population, but have never been described in the pediatric population. This information can better improve patient care and be used to characterize the side effects resultant from these medications.
Sildenafil in Hemodialysis Patients With Pulmonary Hypertension
Sildenafil is a phosphodiesterase inhibitor that can exert a nitric oxide-mediated vasodilation effect, so it's considered one of the preferred agents especially in hypoxia induced pulmonary hypertension, can achieve pulmonary vasodilation by enhancing sustained levels of cyclic guanosine monophosphate (cGMP) and nitric oxide. Despite the potential burden of pulmonary hypertension in hemodialysis patients, such agent like sildenafil has limited studies about optimum dose, safety and long term efficacy in End stage renal disease patients on hemodialysis with pulmonary hypertension
Single Ascending Dose Study for Evaluation of Safety, Tolerability and Pharmacokinetics of L606
The primary objective of this study is to evaluate the Pharmacokinetics, Safety and Tolerability of L606 (Liposomal Treprostinil) Inhalation Solution in Single Ascending Dose study design in healthy volunteers.
Skeletal Muscle Mitochondrial Abnormalities and the Metabolic Syndrome in PAH
Pulmonary arterial hypertension (PAH) is characterized by the progressive increase in pulmonary vascular resistance ultimately leading to right ventricular (RV) failure. Its prevalence is estimated at 40-60 persons per million and predominantly affects people between 20 and 60 years of age. Newly available therapies have improved the 3-year survival to >80%. This improvement in prognosis brings new challenges for clinicians: PAH has changed from a rapidly fatal disease to a chronic disorder with persistent exercise limitation and poor quality of life. Many observations suggest that exercise limitation in PAH is not simply due to...
Sleep-Disordered Breathing in Chronic Thromboembolic Pulmonary Hypertension
There is evidence for a high prevalence of Sleep Disorder Breathing (SDB) in patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Central Sleep Apnea, Cheyne-Stokes Breathing and Obstructive Sleep Apnea appear to occur in CTEPH. However, there is no information on the impact of CTEPH treatment modalities on concomitant SDB. Furthermore, the use of PAP therapy in CTEPH has not yet been investigated. CTEPH is a rare and serious disease and there may be a bidirectional association of SDB and CTEPH. This study plans to investigate the prevalence of SDB in CTEPH and compare it to datasets of large epidemiological studies...
Spironolactone for Pulmonary Arterial Hypertension
Background: - High blood pressure in the lungs, known as pulmonary arterial hypertension (PAH), is a rare disorder. In spite of recent advances in treatment, the death rate remains unacceptably high. Lung blood vessel function can be harmed by progressive injuries, such as inflammation, leading to worsening of the disease. A drug called spironolactone has been known to improve blood vessel function and reduce inflammation. Some people with PAH take spironolactone to help treat fluid retention. However, its effect on inflammation and blood vessel function in patients withPAH is not known. Researchers want to see if spironolactone...
Stress Echo 2020 - The International Stress Echo Study
Background: Stress echocardiography (SE) has an established role in evidence-based guidelines, but recently the breadth and variety of applications has extended well beyond coronary artery disease (CAD). Purpose: To establish a prospective research study of SE applications, in and beyond CAD, also considering a variety of signs in addition to regional wall motion abnormalities. Methods: In a prospective, multicenter, international, observational study design, > 100 certified high-volume SE labs will be networked with an organized system of clinical, laboratory and imaging data collection at the time of physical or pharmacological SE,...
Stress Echocardiography in Patients Recovery From Mild COVID-19 Illness
With the appearance of the new SARS-COV2 virus, additional challenges are being imposed on the medical community after the resolution of acute COVID-19 illness, resulting in specific pathophysiologic mechanisms that while acutely damage the lung parenchyma might chronically impact the cardiopulmonary system. This study aims to investigate changes after mild COVID-19 illness in echocardiographic indices at rest and stress.
Study of Angiogenic Cell Therapy for Progressive Pulmonary Hypertension: Intervention With Repeat Dosing of eNOS-enhanced EPCs
The SAPPHIRE clinical trial seeks to establish the efficacy and safety of repeated monthly dosing of autologous EPCs transfected with human eNOS (heNOS) in patients with symptomatic severe PAH on available PAH-targeted medical therapy.
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