Effect of Morphine on Dyspnea and 6-Minute Walk Distance in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionDespite advances in treatment and corresponding improvements in survival, patients with pulmonary arterial hypertension (PAH) remain highly symptomatic. In one survey of 315 patients with PAH, sixty-eight percent had moderate or severe dyspnea on exertion and 40% had a profound and clinically significant deficit in quality of life. Palliative care is being increasingly investigated in life-limiting cardiovascular diseases to alleviate symptoms. In PAH, its implementation is frequently delayed until end-of-life. Opioids are a common palliative care intervention, however the efficacy and safety of opioids for symptom relief in PAH has not been evaluated.
Goal Oriented Strategy to Preserve Ejection Fraction Trial
Pulmonary Arterial HypertensionIn this prospective long term feasibility study we examine whether a goal oriented therapeutic strategy that is able to preserve right ventricular function will result in improved clinical outcome in patients with pulmonary arterial hypertension. We hypothesize that right ventricular function can only be preserved when early and aggressive medical combination therapy not only reduces pulmonary vascular resistance but also pulmonary pressures.
To Compare the Efficacy of Combination Therapy vs Monotherapy for Pulmonary Arterial Hypertension...
Associated Pulmonary Arterial HypertensionThe study will be carried out on 50 consecutive consenting patients of systemic sclerosis with PAH recruited from outpatient department of internal medicine and rheumatology clinic of PGIMER, Chandigarh, India It is a single centre double blind randomised controlled trial evaluating the effect of upfront dual therapy (sildenafil and bosentan) vs monotherapy (sildenafil) Participants will be randomised in 1:1 ratio to one of treatment arms. Placebo and PDE-5 inhibitors 20 mg BD to 60 mg if patient tolerates the drug well to one study arm and PDE-5 inhibitors plus ER antagonist 62.5 to max of 125 to other study group
A Non-Interventional, Patient Registry For The Collection Of Pre-Defined Safety Data In Patients...
Pulmonary Arterial HypertensionThe Thelin Patient Safety Registry is a post-marketing program in the European Union (EU) that is designed to supplement the reporting of spontaneous adverse events (AE) and better characterize known and potential safety signals for Thelin. The registry is a secure, restricted access, electronic system which collects anonymous, pre-defined, patient-level data on demographic variables, safety monitoring measurements (i.e. liver function tests, haemoglobin and international normalized ratio (INR) measurements), concomitant medications, information on AEs and Thelin drug discontinuation. Regular review of the data is conducted to assess the frequency of identified safety risks and to monitor for the emergence of new safety signals at monthly pharmacovigilance meetings, quarterly signal detection meetings, and for each Periodic Safety Update Report (PSUR).
Iron Deficiency In Pulmonary Hypertension
Idiopathic Pulmonary Arterial HypertensionPatients with idiopathic pulmonary arterial hypertension (IPAH) and iron deficiency were previously shown to have a decreased six-minute walking distance. Therefore the investigators hypothesized that intravenous iron administration would improve exercise capacity in iron deficient IPAH patients. 30 patients will be recruited for iron infusions. At baseline and after 12 weeks (endpoint)exercise test will be performed.
Reversible Secondary Myelofibrosis or Clonal Myeloproliferative Disorder
Primary MyelofibrosisPrimary Pulmonary Hypertension2 moreTo determine the prevalence of myelofibrosis in patients with primary pulmonary hypertension, and to discover if the fibrosis in these patients is primary (AMM) or secondary.
Study of the Initial Combination of Bosentan With Iloprost in the Treatment of Pulmonary Hypertension...
Pulmonary Arterial HypertensionPrevious 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.
Inspiratory Muscle Training in Pulmonary Arterial Hypertension
Pulmonary HypertensionPulmonary arterial hypertension is a rare condition characterised by high blood pressure in the lungs and results in breathlessness and reduced exercise capacity for patients. Previous research has shown weakness in respiratory muscles in these patients that may contribute towards their symptoms. Despite advances in medical therapy, the condition still results in a significant symptom burden. Inspiratory muscle training is a non-invasive intervention involving a device that provides resistance to the muscles of inspiration and increases their strength. This study will investigate the benefit of inspiratory muscle training in patients with pulmonary arterial hypertension who are stable on medical therapy for three months. This will be performed as an outpatient and they will then be reviewed following this with assessment of exercise capacity, breathing capacity (spirometry), quality of life, and assessment of neural respiratory drive (the signals from the brain to the muscles controlling breathing). The study will be based at the Golden Jubilee National Hospital and patients will be recruited from outpatients who are already under the care of the Scottish Pulmonary Vascular Unit.
Resynchronisation Therapy of Right Ventricle in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionPulmonary arterial hypertension is a disease characterised by pathological changes in the pulmonary arteries leading to a progressive increase in pulmonary vascular resistance and pulmonary artery pressure. Right ventricular failure is the main cause of death in patients with pulmonary arterial hypertension, and the ability of the right ventricle to adapt to the progressive increase in pulmonary vascular resistance associated with changes to the pulmonary vasculature in pulmonary arterial hypertension is the main determinant of a patient's functional capacity and survival. Right ventricular dyssynchrony was present in a substantial proportion of patients with pulmonary arterial hypertension and this dyssynchrony adversely affected right ventricular function.
Rehabilitation for Patients With Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionPatients suffering from pulmonary arterial hypertension (PAH) frequently remain symptomatic despite medical therapy. Symptoms include breathlessness, poor exercise capacity and reduced quality of life. In many other serious heart or lung diseases it has been shown that physical rehabilitation improves patient's fitness and quality of life. In PAH there are no clear guidelines and in general physical activity has traditionally been discouraged, although evidence for this advice is lacking. Interesting research project in Germany showed significant benefit for in-patient rehabilitation in PAH patients. In this study we will perform a controlled clinical study of out-patient rehabilitation of patients with PAH. We hypothesize that physical training of patients will result in increased exercise capacity and improved quality of life.