View clinical trials related to Pulmonary Arterial Hypertension.
Filter by:The purpose of this study is to evaluate the safety and effectiveness of an investigational/experimental drug called AIR001. To test the effectiveness, the study will evaluate how AIR001 affects the blood vessels in the lungs and the function of the heart. This will be done by monitoring changes in Pulmonary Vascular Resistance (PVR); from Baseline/Day 1 (start of study drug) to Week 16 of the study. PVR measures the resistance to flow in the blood vessels of the lungs. The study will include other assessments to evaluate the effect of the study drug on PAH, including measurements of exercise ability and evaluations of PAH disease symptoms.
This study will determine the safety and feasibility of using a β-blocker (in this case carvedilol) in the treatment of pediatric patients with Left Heart Failure (LHF) in children with Pulmonary Arterial Hypertension (PAH). Carvedilol affects the nervous system, the same system that is highly activated in response to stress in patients with PAH. Each patient is administered a dosage of carvedilol, according to their weight. This dosage is increased incrementally over the span of the study, if the patient responds well to the drug. The study will determine whether the potential adverse side effects of carvedilol outweigh the possible positive results in reducing LHF. The hypothesis of this study predicts that carvedilol will have positive effects in treating LHF, similar to their use in treatment of Right Heart Failure (RHF). This is a single-centered pilot study. Each patient will be studied for approximately 31 weeks.
6 months therapy of Bosentan, an endothelin antagonist, will lead to improvement in pulmonary microvascular endothelial function.
Previous studies suggest that combinations of existing therapies may be effective for pulmonary arterial hypertension (PAH). However, all of these studies are sequential combination therapy, for example, by adding sildenafil to previously prescribed bosentan. This kind of therapy model is not enough for PAH patients, especially those with New York Heart Association (NYHA) class Ⅲ and Ⅳ. In this randomized, multicenter study, the investigators evaluate the safety and efficacy of combining inhaled iloprost, a prostacyclin analog, with the endothelin receptor antagonist bosentan in treatment naive patients with PAH by comparing with bosentan monotherapy. Efficacy endpoints include change from baseline in 6-min-walk distance (6-MWD), modified (NYHA) functional class, hemodynamic parameters, and time to clinical worsening.
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 can help these conditions in people with PAH. Objectives: - To test the effectiveness of spironolactone in treating pulmonary arterial hypertension. Eligibility: - Individuals at least 18 years of age with pulmonary arterial hypertension. Design: - This study will last for 24 weeks. Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected. - Participants will take either spironolactone or a placebo. They will take their study drug or placebo for 7 weeks. Treatment will be monitored with regular blood tests. - In Week 8, participants who have had no reaction to the treatment will receive a higher dose of the drug or placebo. - In Week 12, participants will have a study visit with heart and lung function tests. They will also have a 6-minute walk test, and provide blood and urine samples. - After additional study visits for blood samples, participants will have a final visit in Week 24. The tests from Week 12 will be repeated at this visit.
Multi-center, observational, U.S.-based longitudinal program. Data will be collected prospectively for 3 years. Individual physician feedback will be provided on data collected with the purpose of improving the management of patients - quality enhancement research initiative (QuERI) process from adult patients enrolled with a history of repaired Congenital Heart Disease (CHD).
Patients with pulmonary arterial hypertension (PAH) suffer from breathlessness, poor quality of life and inability to function, despite medical therapy Current consensus states that combination therapy with different classes of PAH-specific therapy is likely to bring additional benefit to PAH patients. In this study we plan to study how exercise performance changes when the phosphodiesterase inhibitor vardenafil is added to patients who remain symptomatic from PAH when treated with inhaled iloprost. Following baseline assessment, all Patients will start vardenafil 10 mg bid. If the drug is tolerated by the patients, after a two week period, up titration to 20 mg bid will be permitted, at the discretion of the investigators. According to treatment protocol up titration will be done carefully and whenever side effects will be reported up titration will be stopped or dosage will be decreased or stopped according to the investigator judgment. Systemic BP will be measured at baseline assessment. The patient will attend the clinic for the first dose monitoring (10 mg) and after up titration of the study drug to 20 mg. Systemic BP will be measured at baseline assessment. The patient will attend the clinic for the first dose monitoring (10 mg) and after up titration of the study drug to 20 mg. A fall in SBP of>30 mmHg will be considered significant or any smaller value at the discretion of the investigator. BP will be measured according to the following protocol. Pre-dose Immediately before administration of vardenafil. This will be timed approximately one hour prior to the next planned dose of iloprost. Pre-inhalation One hour post vardenafil dose, immediately prior to iloprost inhalation. Post-inhalation Immediately following completion of iloprost inhalation and every fifteen minutes for one hour. Prior to discharge Two hours following the iloprost. Later monitoring At all follow-up visits, BP will be measured. This is an open-label study to evaluate the safety and efficacy of adding higher doses of vardenafil to inhaled iloprost over 3 months.
Mutations in bone morphogenetic protein receptor 2 (BMPR2) are present in >80% of familial and ~20% of sporadic pulmonary arterial hypertension (PAH) patients. Furthermore dysfunctional BMP signaling is a general feature of pulmonary hypertension even in non-familial PAH. We therefore hypothesized that increasing BMP signaling might prevent and reverse the disease. We screened > 3500 FDA approved drugs for their propensity to increase BMP signaling and found FK506 (Tacrolimus) to be a strong activator of BMP signaling. Tacrolimus restored normal function of pulmonary artery endothelial cells, prevented and reversed experimental PAH in mice and rats. Given that Tacrolimus is already FDA approved with a known side-effect profile, it is an ideal candidate drug to use in patients with pulmonary arterial hypertension. The aims of our trial are: 1. Establish the Safety of FK506 in patients with PAH. 2. Evaluate the Efficacy of FK506 in PAH 3. Identify ideal candidates for future FK506 phase III clinical trial.
The purpose of this study is to determine if cardizem is effective in the treatment of nitric oxide non-responder pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is a rare, progressive, and life-threatening disease. In many patients, the course of PAH is a steady deterioration and reduced life expectancy. Sildenafil was approved by the European Commission for the treatment of PAH in pediatric patients in May 2011, making it the first agent to be approved for the treatment of children with PAH. The approval was based on the largest placebo-controlled study to be conducted in this population. The recommended dose in pediatric patients aged 1 year to 17 years old is 10 mg TID in patients ≤ 20 kg and 20 mg TID for patients > 20 kg. Higher doses are not recommended in pediatrics patients. This study is an open-label, multi-center study to investigate safety, efficacy and pharmacokinetics of sildenafil citrate in Japanese pediatric patients with PAH.