
Ambrisentan in Patients With Porto-pulmonary Hypertension A Multicenter Open Label Trial
Portopulmonary HypertensionThis is an Open Label, Multicenter, pilot clinical trial to assess the efficacy and safety of an oral selective Endothelin Receptor Antagonist (ambrisentan) in patients with portopulmonary hypertension. Preliminary evidence suggests that ambrisentan is safe and effective in patients with portopulmonary hypertension. The goal of therapy for these patients is to improve symptoms of dyspnea and to improve pulmonary hemodynamics to a mean pulmonary artery pressure <35 mm Hg in order to make patients eligible for liver transplantation. Therefore, the primary endpoints for this study will include 6 minute walk distance (6MWD) and pulmonary vascular resistance (PVR). Eligible subjects will receive 5 mg ambrisentan once-daily for the first 4 weeks. After the initial 4-week period, investigators will increase study drug dose to 10 mg once daily (both 5 mg and 10 mg doses are FDA approved). If 10 mg is not tolerated in the opinion of investigator, then the investigator may decrease the dose back to 5 mg once daily. Primary outcome is a change in both the 6 Minute Walk Distance and in Pulmonary Vascular Resistance from baseline to Week 24. Subjects will be monitored with liver function tests (LFT) every 2 weeks for the first 8 weeks, then every 4 weeks thereafter. These safety laboratory tests may be performed at a local phlebotomy laboratory or at the Investigator clinic. In addition, the Investigator will assess each subject for safety and efficacy at Week 4, Week 12, and Week 24. Following Week 24, subjects will be assessed for safety and efficacy every 12 weeks. Patients will be followed for a total of 1 year. After 1 year, if the Investigator feels that continuing the treatment will be beneficial to the patients, they will be provided with ambrisentan by Gilead Pharmaceuticals, free of charge.

Long-term Ambrisentan Extension Study for Pediatric Patients Who Participated in AMB112529
HypertensionPulmonaryAn open label, long term extension to Study AMB112529. All subjects may remain in the extension study for a minimum of six months. Beyond the six month period, subjects may continue in the extension study until one of the following conditions is met: the subject turns 18 years of age (when the subject can receive marketed product) the product is approved and available for use in the subject's age group, development for use in the paediatric population is discontinued. the subject decides he/she no longer wants to participate in the study, the investigator considers it is in the best interest of the subject to discontinue ambrisentan (e.g. for safety reasons). The primary objective is the long-term safety and tolerability of ambrisentan in the paediatric PAH population. Secondary objectives are all cause mortality and change from baseline in Study AMB112529 on efficacy parameters.

Exercise Induced Pulmonary Hypertension in Systemic Sclerosis and Treatment With Ambrisentan
Systemic SclerosisShortness of Breath1 moreThe purpose of this study is to determine the clinical characteristics and hemodynamic profiles that predict exercise induced pulmonary hypertension in 15 patients with systemic sclerosis. The study also aims to determine the effectiveness of Ambrisentan for subjects with exercise induced Pulmonary Arterial Hypertension (PAH) with scleroderma

Atorvastatin in Pulmonary Hypertension
HypertensionPulmonaryClinical effect and tolerability of atorvastatin versus placebo in patients with Pulmonary Hypertension: double-blinded, randomised, prospective phase II study for 6 months with adjusted doses of Atorvastatin

Sildenafil IV Bolus Study
HypertensionPulmonaryThe objective of this study is to assess the safety, tolerability and pharmacokinetics of intravenous sildenafil (10 mg) administered as a bolus injection to patients with Pulmonary Arterial Hypertension already receiving and stable on oral Revatio 20 mg TID.

BAY63-2521 - Long-term Extension Study in Patients With Chronic Thromboembolic Pulmonary Hypertension...
Pulmonary HypertensionPatients who have completed the 16 weeks treatment of the CHEST-1 trial (study number 11348) will be asked to participate in this long term extension study with BAY63-2521. The aim of the long term study is to collect additional information to evaluate the safety and tolerability of BAY63-2521. Patients will be treated with open label medication on their individual optimal dose between 0,5 mg - 2,5 mg tid.

BAY63-2521:Long-term Extension Study in Patients With Pulmonary Arterial Hypertension
HypertensionPulmonaryPatients who have completed the 12 weeks treatment of the PATENT-1 trial (study number 12934) will be asked to participate in this long term extension study with BAY63-2521.

The Impact of Udenafil on Exercise Capacity in Severe Chronic Obstructive Pulmonary Disease (COPD)...
DyspneaChronic Obstructive Pulmonary Disease1 morePulmonary hypertension (PH) is a serious complication of COPD which is associated with shorter survival, more frequent exacerbation, and increased use of health resources. There is no effective pharmacological treatment for COPD-associated PH. Therefore, the investigators wanted to evaluate the effect of udenafil, a phosphodiesterase- 5 (PDE-5) inhibitor, on exercise capacity of severe COPD patients.

Iloprost in Gas Exchange/Pulm Mechanics in Chronic Obstructive Pulmonary Disease (COPD)
Chronic Obstructive Pulmonary DiseasePulmonary HypertensionThe investigators believe that iloprost will improve gas exchange in COPD patients with pulmonary hypertension.

Inhaled Iloprost for Sarcoidosis-associated Pulmonary Hypertension
SarcoidosisPulmonary Arterial HypertensionThis trial will study the treatment of sarcoidosis-associated pulmonary arterial hypertension with inhaled iloprost, a drug approved for primary pulmonary arterial hypertension.