Active clinical trials for "Familial Primary Pulmonary Hypertension"

Prospective, multi-center, open-label, single-arm, Phase 3b extension study of macitentan in patients with PAH. To assess the long-term safety of macitentan in patients with pulmonary arterial hypertension (PAH) beyond treatment in the AC-055-310 study.

The purpose of this study is to see if Lung LLC's new experimental formulation of the medicine Beraprost Sodium, called Beraprost Sodium 314d Modified Release (BPS-314d-MR), can improve the symptoms of pulmonary arterial hypertension (PAH) in patients. An experimental drug is one that has not been approved by the U.S. Food and Drug Administration for use in the general public. This research study is for patients who have pulmonary arterial hypertension (PAH) and have just completed taking part in an earlier research study and received an older experimental formulation of Beraprost Sodium, called Beraprost Sodium Modified Release (BPS-MR). That earlier study was being done to see if BPS-MR could improve their PAH. Patients may also be taking Tyvaso (treprostinil), Tracleer (bosentan), Letairis (ambrisentan), Adcirca (tadalafil) and/or Viagra or Revatio (sildenafil) to treat their PAH. The diagnosis of PAH means that the blood pressure in their lungs is higher than normal. The increased blood pressure in the lungs places a strain on the heart. The strain causes the heart to pump less blood into the lungs, causing shortness of breath and tiredness. The strain on the heart weakens the heart muscle making it less able to pump blood, a condition called heart failure. As heart failure develops, swelling in the feet and abdomen may occur.

The study was conducted as a placebo-controlled, randomized, 22-week double-blind study which included a dose titration period. An additional transition period occurred for those patients who elected to enroll into the open-label extension study, APD811-007. A total of 61 patients with PAH were enrolled.

This study evaluates the long-term safety of Udenafil in Patient with Pulmonary Arterial Hypertension(PAH). All the patients will receive Udenafil for 1-year(48-weeks).

The purpose of this study is to Determine blood peptide levels and investigate any tissue gradients of apelin in patients with idiopathic pulmonary arterial hypertension and healthy volunteers. Evaluate the effect of exogenous apelin infusion on exercise performance in healthy volunteers and patients with idiopathic pulmonary arterial hypertension.

This is an international, multicenter, randomized, double-blind, placebo-controlled, event driven study in subjects with pulmonary arterial hypertension.

This study sought to determine whether terbogrel was an effective treatment for primary pulmonary hypertension (PPH) by comparing its efficacy and safety to that of placebo.

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.

A Phase 2 open label, dose escalation study to find the minimally and maximum effective dose (dose beyond which no further effect on PVR is seen) of inhaled nitric oxide generated by the GeNOsyl® System compared to placebo.

This multi-center, open-label study will assess the tolerability and safety of transitioning subjects with stable Pulmonary Arterial Hypertension (PAH) from continuous intravenous (IV) or subcutaneous (SC) Remodulin infusion to oral treprostinil (UT-15C sustained release (SR) tablets). This study will consist of an in-hospital transition phase, dose optimization/evaluation phase, and follow up phase.