Study of Cardiac MRI in the Follow up Assessment of Patients With PAH (EVITA)
Pulmonary arterial hypertension (PAH) is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular (RV) failure and eventually to death. The therapeutic strategy has become complex and needs to perform recurring follow up evaluations including a right heart catheterization (RHC). Cardiac magnetic resonance imaging (cMRI) has the advantage to accurately assess right ventricular (RV) volumes and important prognostic predictors such as cardiac index, stroke volume (SV) and right ventricular ejection fraction (RVEF). The main objective is to assess the hemodynamic diagnosis performances...
Subcutaneous Elafin in Healthy Subjects
A multiple-ascending-dose (MAD), randomized, placebo-controlled, blinded trial to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of Elafin in healthy adult subjects. The purpose of this study is to assess Elafin that is being developed for treatment of PAH. Elafin inhibits elastase, an enzyme that is increased in pulmonary hypertension and is a major factor in the development of PAH. Elafin will be administered subcutaneously daily for 7 days in normal healthy subjects followed over a 28 day time period.
Symptom-driven Referral for Evaluation of Chronic Thromboembolic Disease or Pulmonary Hypertension in Patients With Previous Acute Pulmonary Embolism
Aim: To investigate if a symptom driven referral for chronic thrombosis in the lungs after acute pulmonary embolism is better than the current approach. Background: A number of patients with chronic thrombosis in the lungs after acute pulmonary embolism have dyspnea and reduced functional capacity without elevated pulmonary arterial pressure at rest (CTED). However, current guidelines for follow-up after acute pulmonary embolism will miss all patients with CTED, as referral for further examination is based on elevated pulmonary arterial pressure on echocardiography. Thus, the prevalence of CTED is unknown. The hypothesis is, that a...
Symptom-related Screening for Early Detection of CTEPH.
Routine screening for Chronic thromboembolic pulmonary hypertension (CTEPH) i after PE is not supported by current evidence and guidelines. This study aims to evaluate if a screening program for patients with acute PE based on telephone monitoring of symptoms and further examination if only symptomatic patients could help an early detection of CTEPH.
Taiwan Cohort - Chronic ThromboEmbolic Pulmonary Hypertension Registry
The diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) is difficult for numerous reasons and is related with a poor prognosis. In Taiwan, the incidence of CTEPH and its clinical features are unknown. This study aims at evaluating the prevalence, clinical characteristics, and management of CTEPH in a Taiwanese cohort. Primary objective: - To investigate the geodemographics of CTEPH in Taiwan Secondary objectives: - To characterize the demographics and clinical presentation of patients with CTEPH - To describe the real-world management and treatment outcome of patients with CTEPH - To...
Tamoxifen Therapy to Treat Pulmonary Arterial Hypertension
The main purpose of this clinical trial is to examine the feasibility and effects of tamoxifen in subjects with pulmonary arterial hypertension (PAH). The study will evaluate how well the drug is tolerated, and its impact on functional condition and selected biomarkers. Changes in tricuspid annular plane systolic excursion (TAPSE) and other parameters determined by transthoracic echocardiography will be evaluated as well as changes in additional metrics such as six minute walk test distance, quality of life assessments, and hormone levels.
The Effect of Oxygen Therapy on 6MWD in PAH and CTEPH Patients With Hypoxemia
Treatment of O2 naïve patients with PAH will be included in this investigator-initiated trial (IIT) to assess efficacy and safety of oxygen substitution. Nocturnal oxygen substitution improved the 6MWD compared to placebo in one clinical trial in PAH patients. Due to the positive results in the treatment of patients with PAH, the initiation of this proof-of-concept study is justified.
The Effects of Commercial Air Travel on Patients Suffering From Pulmonary Hypertension
The aim of this study is to 1) descriptively report possible in flight events and 2) to provide regression analysis if the number of events are statistically significant in their prevalence and thus are useful in finding possible parameters in echocardiography, right heart catheterization, laboratory findings, spiroergometry as well as six minute walk test to produce a risk assessment for possible expected in flight adverse events as well as a recommendation concerning the need of supplemental oxygen for each individual patient. The investigators therefore want to find out: 1. In which subgroup (if applicable) of PH patients in...
The PAH Platform for Deep Phenotyping in Korean Subjects
A total of 16 regional hospitals will be registering clinical data and biological specimens of idiopathic pulmonary arterial hypertension (IPAH)/heritable pulmonary arterial hypertension (HPAH) patients across Korea. The diagnosis of pulmonary arterial hypertension(PAH) will be based on right heart catheterization, where PAH caused by etiology other than HPAH or IPAH will be excluded. All clinical data will be stored to a government-based online database. Each participating hospitals will be collecting whole blood from each patient, through which DNA, RNA, serum, plasma, and peripheral blood mononuclear cells will be extracted from...
The Prevalence of Pulmonary Hypertension in Patients With COVID-19.
The virus infection Covid-19 fills our hospitals and intensive care departments in a very unique way and there is a lack of essential insight into the pathophysiology of the disease. As a result, very specific treatment options are missing. The US Medicines Agency (FDA) has in the last days given a general license for treatment with inhaled nitric oxide (iNO). Inhaled NO in Sweden (and Europe) is approved for the indication of pulmonary hypertension in adults. However, no one has yet described the occurrence of pulmonary hypertension, with or without right ventricular loading, in the Covid-19 patients who become so seriously ill...
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