Multi-center, Web Based Observational Study of Pulmonary Hypertension in Scleroderma Patients
Systemic SclerosisScleroderma2 moreThe purpose of this study is to determine the timeline of progression from pre-pulmonary hypertension to diagnosable pulmonary hypertension based on right heart catheterization. Moreover, to determine the timeline for progression from diagnosable pulmonary hypertension to clinical worsening of disease as defined as death, hospitalization, or worsening of PHT symptoms.
Drug Use Investigation for VOLIBRIS® (Ambrisentan) (Pulmonary Arterial Hypertension)
HypertensionPulmonaryThe objective of this post-marketing surveillance study is to evaluate the incidence of adverse events in Japanese subjects with pulmonary arterial hypertension treated with ambrisentan basd on prescribing information under the conditions of general clinical practice and also to grasp the following items; Unknown adverse drug reactions (ADRs) Incidence of ADRs to medical products in actual clinical practice Factors influencing safety of ambrisentan Factors influencing efficacy of ambrisentan Prognosis of subjects as well as efficacy and safety of ambrisentan in long-term use (VOLIBRIS® is a trademark of Gilead Sciences, Inc,. that GSK uses under license.)
Observation of Patients With Primary Pulmonary Hypertension Receiving Prescribed Ventavis Inhalation...
HypertensionPulmonaryThis is an observational study to monitor the continued effectiveness of Ventavis (inhaled iloprost) in the long-term. The study observes the effects and the safety of Ventavis inhalation therapy over at least 2 years and up to 4 years. A total of 54 patients from around 30 study sites in Europe will be included in the study. This observational study will collect information in patients receiving a medication that is already available on prescription in the participating countries. Ventavis is used to treat moderate cases of primary pulmonary hypertension.
BMPR2 Mutations and Iron Metabolism in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionPreviously characterised PAH patients, including idiopathic, heritable and other forms of group 1 PAH with and without BMPR2 mutation which have already been analysed and are regularly seen in the Center for Pulmonary Hypertension may be contacted to participate in the study. Clinical and laboratory values will be collected prospectively. Patients with IPAH/HPAH and other forms of PAH who are newly diagnosed within the duration of the trial will receive routine diagnostic workup including the routine information about a possible BMPR2 mutation analysis for IPAH/HPAH patients according to guidelines. During their routine visit the patients' medical history will be obtained and physical examination will be conducted. Moreover, an electrocardiogram (ECG), determination of World Health Organization (WHO)-functional class, laboratory testing (NT-proBNP and routine laboratory), echocardiography will be routinely carried out. BMPR2 expression levels will be measured in blood samples. Additionally, laboratory samples will be collected for analysis of further parameters reflecting iron metabolism such as hepcidin, ferritin, iron levels, IL6 and circulating soluble transferrin receptor Levels. In addition, healthy controls will be invited to participate in this study to obtain comparable levels of hepcidin and BMPR2 pathway members.
a Non-interventional Study of Sildenafil in the Treatment of Pulmonary Arterial Hypertension (PAH)...
Pulmonary Arterial Hypertension (PAH)This observational study will involve analysis of data collected by the National Rare Disease Registry or medical records .The study will describe outcomes only in PAH(Pulmonary arterial hypertension) patients treated with sildenafil; there will be no comparison with another treatment group. Approximately 100 adults with PAH will be recruited in China hospitals This NIS(non-interventional study) data will be recorded by a physician in the medical records, during the patients' clinical visits, and in the electronic Case Report Forms (CRF).
Chronic Thrombo-embolic Pulmonary Hypertension: Classification and Long Term Outcome
Chronic Thromboembolic Pulmonary HypertensionIdiopathic Pulmonary Arterial HypertensionThis study will help to provide more up to date prognostic data on the natural history of untreated proximal CTEPH which will be helpful in discussions regarding surgical versus medical treatment and by exploring the similarities and differences between distal CTEPH and IPAH the investigators hope to get insights into disease mechanisms in patients with distal CTEPH.
Evaluate The Clinical Effectiveness, Safety And Tolerability Of Sildenafil Used In Doses ≥20mg TID...
Pulmonary Arterial HypertensionThe objective of this observational study is to gain clinical insight on the actual use of sildenafil citrate (Revatio™) for the treatment of pulmonary arterial hypertension (PAH). The primary objective is to assess effectiveness and safety of sildenafil at doses ≥20mg three times daily for the treatment of PAH.
Rt Ventricular Substrate Metabolism as a Predictor of Rt Heart Failure in Patients With Pulmonary...
Pulmonary Arterial HypertensionThe purpose of this study is to evaluate patterns of metabolic activity in the heart of patients with pulmonary arterial hypertension(PAH). Patients with PAH are at risk of developing weakness or failure of the right side of the heart.It is possible that there is a relationship between the development of heart failure and the way the heart uses energy sources, such as sugar. This study is designed to evaluate the way the heart uses sugar uptake in patients with PAH using positron emission tomography(PET imaging)
Study Of Right Ventricular Performance In PAH Patients Treated With Rapid Dose Treprostinil (Remodulin)...
Pulmonary Arterial HypertensionPatients with pulmonary arterial hypertension (PAH) are at much higher risk of death if the RV (right ventricle) is weak. The purpose of this study is to get a better understanding of the factors that determine RV adaptation and how the RV compensates on therapy. The investigator is also interested in how Remodulin (treprostinil) infused over a short period (approximately 48-72 hours) affects the patient's quality of life, medical care, and personal health behaviors. Treprostinil, also known as Remodulin, has been approved by the US Food and Drug Administration for use in the treatment of PAH. The investigator has been treating patients with Remodulin by rapid infusion (over 48 hours) for over 6 years. The investigator would like to establish this practice as safe and effective for the benefit of other centers that treat PAH.
A Pharmacokinetic Substudy of the TDE-PH-304 Protocol
Pulmonary Arterial HypertensionA sub-study to the TDE-PH-304 protocol to assess the pharmacokinetics of patients transitioning from a twice daily dosing regimen of oral treprostinil to a three times daily dosing regimen.