Active clinical trials for "Pulmonary Arterial Hypertension"

The purpose of this study is to assess tolerability and clinical effects of transition from intravenous (IV, needle in the vein) or subcutaneous (SQ, needle in the skin) to the recently-approved inhaled treprostinil (Tyvaso) for the treatment of pulmonary arterial hypertension (PAH). Our hypothesis is that the transition to inhaled treprostinil will be tolerated by patients. The intravenous and subcutaneous drugs epoprostenol and treprostinil received approval for treatment of PAH many years ago. While these medications improve exercise capacity and the symptoms of PAH, they are given by injection and thus have several side effects, such as pain and catheter infection. This has resulted in many patients either refusing to take the medication or quitting these medications because of not tolerating them. The only other form of prostacyclin treatment available for PAH patients is inhaled. There are 2 inhaled prostacyclins approved for PAH, however one of these requires at least 6 inhalations per day, every day, and takes about 30 minutes to inhale each time. Thus, it has not been a regularly-used medication and issues surrounding compliance make it a riskier drug to use if patients do not get their full doses every day. The other inhaled medication, treprostinil, was approved a few months ago, only needs to be given 4 times a day, and takes about 2-3 minutes to inhale. Since inhaled treprostinil can be administered easily, it is anticipated that many patients will transition from epoprostenol or treprostinil to the recently approved inhaled treprostinil, however we do not know if this is a safe or effective way to manage patients. Thus, the goal of this prospective study is to gather observational data regarding how that switch is made, tolerability of the switch, and, to the extent possible with this methodology, assess clinical effects of the switch. This is a prospective study. Twenty patients > 18 years old with PAH will be enrolled. Patients enrolled will be those in whom a clinical decision to convert from either IV epoprostenol, IV treprostinil, or SQ treprostinil to inhaled treprostinil therapy has been made. This is usually the result of patients asking to switch to inhaled therapy, but only allowed by physicians if they feel the switch would be safe. If eligible, and after informed consent, patients will have a history and physical examination, a 6 min walk test, a cardiopulmonary exercise test (CPET), blood tests, and a symptom questionnaire will be filled out. Patients will then be admitted to the hospital where a monitoring catheter will be placed inside the patient's heart and inhaled treprostinil will be initiated, while the dose of IV/SQ medication is reduced over about 24-26 hours. Clinical follow-up will be at weeks 1, 4, and 12. The procedures above are all part of the routine clinical care that patients would receive if they were to be transitioned to inhaled therapy, including the hospitalization and catheterization. The criteria for them to be able to be switched are conservative. Pressure in their heart and lungs must be low (mPAP < 40 mmHg and RAP <12 mmHg on catheterization), and their dose of IV or SQ medication must be low (< 20 ng/kg/min). Regarding the patient subset enrolled in this study in whom a clinical decision to convert transition therapy has been made, we will try to ensure that our clinical decision-making will not be influenced by the need to enroll subjects in the study by explicitly noting the potential for conflict of interest with each patient (addressed in the ICF). We will not make a clinical decision for our patients based on the desire to fill the study numbers, and every will be made to avoid the potential for a perceived conflict of interest.

The AC-065A302 (GRIPHON) study is an event-driven Phase 3 study to demonstrate the effect of selexipag on time to first morbidity or mortality event in patients with pulmonary arterial hypertension.

Exercise-induced increase of the pulmonary arterial pressure may be an early sign of pulmonary arterial hypertension. It has been shown that patients with normal pulmonary arterial pressure at rest but elevated pulmonary arterial pressure during exercise have a decreased exercise-capacity and may have a worse prognosis compared to patients with normal pulmonary arterial pressure values at rest and during exercise. According to the currently used definition pulmonary hypertension can be diagnosed if the mean pulmonary arterial pressure is higher than 25mmHg at rest or 30mmHg during exercise. In this study patients with a risk for pulmonary arterial hypertension (connective tissue disease) and increased pulmonary arterial pressure values during exercise are receiving a therapy with a dual endothelin receptor antagonist - bosentan, a therapy established for pulmonary arterial hypertension. The therapy effect is than compared to the recorded changes before the introduction of this therapy.

The primary objective of this study is to evaluate the effect of GSK1325760A on improvement in exercise capacity in subjects with pulmonary arterial hypertension (PAH). The secondary objectives of this study are to evaluate administration of GSK1325760A on: The safety and tolerability Improvement of PAH The steady-state plasma pharmacokinetics of GSK1325760A

To evaluate the change from baseline in pulmonary vascular resistance (PVR), and other hemodynamic parameters, following the addition of ambrisentan to background phosphodiesterase type-5 inhibitor (PDE-5i) therapy in subjects with pulmonary arterial hypertension (PAH) who have demonstrated a sub-optimal response to PDE-5i monotherapy. The study was originally designed as a 2-arm, double-blind, randomized study in which patients received ambrisentan or placebo for 24 weeks, and then received ambrisentan blinded to dose for 24 weeks. With Protocol Amendment 2 (12 June, 2009), the study was switched to single-arm, open-label treatment, and all patients remaining in the placebo arm were switched to open-label ambrisentan treatment. Patients who enrolled after Amendment 2 all received open-label ambrisentan.

AMB-320/321-E was designed to provide long-term, controlled monitoring of pulmonary arterial hypertension (PAH) patients treated with ambrisentan (AMB) in order to properly define the adverse event profile associated with this endothelin receptor antagonist (ERA), including the incidence and severity of elevated serum liver function tests (LFTs). In addition, this study continued the efficacy assessments of the previous studies, examined long-term AMB treatment success, and compared long-term survival of subjects treated with AMB to the NIH registry of patients with PAH.

To evaluate the effect of three doses of oral sildenafil (20, 40 and 80 mg three times a day [TID]) on exercise capacity, as measured by the 6-Minute Walk test, as well as the safety and tolerability, after 12 weeks of treatment in subjects with pulmonary arterial hypertension who are aged 18 years and over. To investigate the plasma concentration-effect relationship and to determine the population pharmacokinetic (PK) parameters.

This is a four-part study of the safety, tolerability, and PK profile of sodium nitrite inhalation solution (AIR001) of ascending multiple doses (Part A) and of escalating doses with steady-state sildenafil (Part B) to healthy male and female subjects, as well as assessment of the safety and tolerability of multiple doses of AIR001 to patients with pulmonary arterial hypertension (part C) with a single dose PK study of AIR001 utilizing three different nebulizers (Part D).

A phase 3, randomized, double-blind, placebo-controlled study to assess safety and efficacy of ambrisentan in subjects with pulmonary arterial hypertension.

To assess the efficacy and safety of sildenafil when added to patients with PAH who are taking bosentan as all or part of their background therapy.