Active clinical trials for "Pulmonary Arterial Hypertension"

This research study is looking at the use of the drug ambrisentan and if it can improve right ventricle function in people with systemic sclerosis-associated pre-pulmonary arterial hypertension. It is also looking at using right ventricle function changes as a marker of disease severity.

Long-term study to evaluate if macitentan is safe, tolerable and efficient enough to be used for treatment of Eisenmenger syndrome.

Treprostinil subcutaneous (under the skin) infusion is a very good medication for treating pulmonary arterial hypertension but infusion site pain may be very severe in some patients. The investigators plan to treat patients receiving treprostinil with a subcutaneous infusion of lidocaine (a local anesthestic) to treat the pain.

Multi-center, double-blind, placebo-controlled, randomized, parallel study comparing continuous intravenous (IV) Remodulin® to placebo in patients with pulmonary arterial hypertension either primary (PPH) or associated with human immunodeficiency virus (HIV) infection or collagen vascular disease).

The purpose of this study is to characterize the clinical and hemodynamic response of Pulmonary Arterial Hypertension (PAH) therapy in patients with atypical PAH and risk factors for left heart disease.

To demonstrate a dose response for 1 mg, 5 mg and 20 mg TID oral sildenafil for the treatment of subjects with PAH.

The investigators will conduct a, randomized, phase 2, placebo-controlled, double-blinded, crossover trial of carvedilol in 26 PAH patients with World Health Organization functional class II or III symptoms and RV ejection fraction (EF) < 45% for 6 months.

To determine whether changes in endothelial cell dysfunction are associated with changes in total pulmonary resistance in patients with pulmonary arterial hypertension

Pulmonary hypertension is characterized by an increase in the pressures in the blood supply to the lungs greater than a mean pressure of 25mmHg and a concomitant increase in overall pulmonary vascular resistance (PVR). In patients who have remodeling of their pulmonary vasculature, PVR will increase with exercise instead of decreasing as it would in normal patients. Based on published evidence, the investigators intend to investigate the effects of inhaled nitric oxide (iNO) on patients undergoing standard exercise techniques who have separately and previously had an implanted pulmonary artery monitoring device (CardioMems by St Jude Medical, Inc.) placed.

Exercise capacity (EC) is limited in pulmonary arterial hypertension (PAH) by impaired right ventricular (RV) function and inability to increase stroke volume (SV). Disease targeted therapy, increases EC by improving SV. Additional factors may contribute to exercise limitation: Peripheral and respiratory muscle dysfunction Autonomic dysfunction An altered profile of inflammation Mitochondrial dysfunction. The enhancement of EC achieved pharmacologically may therefore be limited. Exercise training in PAH improves EC and quality of life (QOL). The changes in physiology responsible for this improvement are not clear. Patients with PAH stable on optimal oral therapy, but not meeting treatment goals, will be enrolled in a 30-week randomised exercise training program. One arm will undertake training for 15 weeks (3 weeks residential, 12 outpatient), the other will receive standard care for 15 weeks then 15 weeks training. Aims: Demonstrate that exercise training can enhance EC and QOL when added to optimal drug therapy a UK PAH population. Explore mechanisms of exercise limitation and factors that improve with training, assessing: Cardiac function Skeletal muscle function Autonomic function Respiratory muscle strength Serum and muscle profile of inflammation Primary outcomes (15 weeks) 6 minute walk distance QOL RV ejection fraction