Active clinical trials for "Pulmonary Arterial Hypertension"

The primary objectives of the study are to evaluate the safety and tolerability, and pharmacokinetics (PK) of sotatercept over 24 weeks of treatment in children ≥1 to <18 years of age with PAH World Health Organization (WHO) Group 1 on standard of care (SoC). There is no formal hypothesis.

Pulmonary Arterial Hypertension is characterized by a progressive increase in pulmonary vascular resistance inducing shortness of breath and exercise intolerance. We aim to correlate cardiac function (evaluated at rest by right heart catheterism and RMN) to exercise capacity (evaluated by endurance time at 75% of maximal workout), in prevalent patients with pulmonary arterial hypertension, and their evolution at three and twelve months.

Background: For patients with out-of-hospital cardiac arrest (OHCA) at the intensive care unit (ICU), oxygen therapy plays an important role in post resuscitation care. During hospitalisation, a lot of these patients occur with pulmonary arterial hypertension (PAH). Currently a wide oxygen target is recommended but no evidence regarding optimal treatment targets to minimise the prevalence of PAH exists. Methods: The RELIEPH trial is a substudy within the BOX (Blood pressure and OXygenation targets in post resuscitation care) trial. It is a single-center, parallel-group randomised controlled clinical trial. 300 patients with OHCA hospitalised at the ICU are allocated to one of the two oxygenation interventions, either a restrictive- (9-10 kPa) or liberal (13-14 kPa) oxygen target both within the recommended range. The primary outcome is the fraction of time with pulmonary hypertension (mPAP >25 mmHg) out of total time with mechanical ventilation. Secondary outcomes are: length of ICU stay among survivors, lactate clearance, right ventricular failure, 30 days mortality and plasma brain natriuretic peptide (BNP) level 48 hours from randomisation. Discussion: This study hypothesises that a liberal target of oxygen reduces the time with PAH during mechanical ventilation compared to a restrictive oxygen target in patients with OHCA at the ICU. When completed, this study hopes to provide new knowledge regarding which oxygen target is beneficial for this group of patients.

Background: A heart catheterization is a diagnostic heart procedure used to measure pressures and take pictures of the blood flow through the heart chambers. Magnetic resonance imaging (MRI) fluoroscopy shows continuous pictures of the heart chambers that doctors can watch while they work. Researchers want to test this procedure with catheterization tools routinely used in x-ray catheterization called guidewires. Guidewires will help move the heart catheter through the different heart chambers. Guidewires are usually considered unsafe during MRI because MRI can cause a guidewire to heat while inside the blood vessels and heart. Researchers are testing special low energy MRI settings that allow certain guidewires to be used during MRI catheterization without heating. Using these guidewires during MRI may help to decrease the amount of time you are in the MRI scanner, and the overall time the MRI catheterization procedure takes. Objectives: To test if certain MRI settings make it safe to use a guidewire during MRI fluoroscopy. Eligibility: Adults 18 and older whose doctors have recommended right heart catheterization. Design: Researchers will screen participants by reviewing their lab results and questionnaire answers. Participants may give 4 blood samples. Participants will be sedated. They will have a tube (catheter) placed in the groin, arm, or neck if they don t already have one. Patches on the skin will monitor heart rhythm. Special antennas, covered in pads, will be placed against the body. Participants will lie flat on a table that slides in and out of the MRI scanner as it makes pictures. Participants will get earplugs for the loud knocking noise. They can talk on an intercom. They will be inside the scanner for up to 2 hours. They can ask to stop at any time. During a heart catheterization, catheters will be inserted through the tubes already in place. The catheters are guided by MRI fluoroscopy into the chambers of the heart and vessels. The guidewire will help position the catheter.

Pulmonary arterial hypertension (PAH) is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and eventually to death. The therapeutic strategy has become complex and needs to perform recurring follow up evaluations including right heart catheterizations (RHC). Cardiac magnetic resonance imaging (cMRI) has the advantage to accurately assess right ventricular volumes and important prognostic predictors such as cardiac index, stroke volume and right ventricular ejection fraction. The main objective of EVITA is to assess the hemodynamic diagnosis performances at baseline and at follow up visits of cMRI in comparison with the results of the RHC (current guidelines) to detect an unfavorable hemodynamic status. The primary endpoint is sensitivity and specificity of cMRI for the diagnosis of an unfavorable status defined by the current RHC criteria (with 95% confidence interval). The secondary objectives are 1) to identify clinical and hemodynamic variables independently contributing to prognosis, 2) to describe complications due to cMRI and to RHC, 3) to compare acceptability and tolerability of cMRI over RHC for the patient and 4) to constitute biological collection of blood samples to determine diagnostic and prognostic PAH biomarkers. PAH patients will be recruited in centers of the French network of severe pulmonary hypertension in a prospective cohort study. 180 subjects will be enrolled in the study: that size will give the study 90% power to find significant at the 5%-level. If the primary endpoint were achieved, since first, strategies and procedures planed in this project are consistent with those currently used in routine and second, inclusion criteria are not limited to a sub-population of PAH patients, positive results could allow to broadly extend our findings. Therefore, it will be possible to decrease the number of RHC, an invasive and cumbersome procedure without altering the prognosis. Moreover, all clinical procedures would be performed in outpatient clinics and thereby would reduce the cost to assess the severity of the disease. Current recommendations for evaluation of severity and follow-up being mainly derived from consensus of opinion of the experts, positive results will also improve the level evidence of severity assessment of PAH patients. According to secondary objectives we expect to better predict morbimortality events with cMRI compared to RHC.

Study ROR-PH-303, ADVANCE EXTENSION, is an open-label extension (OLE) study for participants with WHO Group 1 PAH who have participated in another Phase 2 or Phase 3 study of ralinepag.

Pulmonary arterial hypertension (PAH) is mortal disease affecting the blood vessels of the lung. Despite its morbid prognosis, PAH is often misdiagnosed or ignored, with an average time of 44 months between onset of symptoms to diagnosis and substantial progression of disease severity. Therefore, a pressing need exists to develop non-invasive diagnostic imaging tools, particularly that can detect early disease stages. Efforts have been made to develop such imaging capabilities through platform development of echocardiography, cardiac MRI, chest computed tomography (CT), and positron emission tomography (PET), among others. While some have demonstrated promise, few have shown a precise ability to offer disease quantifications of the diseased lung and vasculature itself, to detect early stages of disease, and to reflect alterations of the lung, vasculature, and right ventricle that reflect the molecular origins of this disease. [F-18]FGln has been previously utilized in oncology studies as a non-invasive in vivo imaging biomarker of tumor glutamine flux and metabolism. Our preliminary in vivo pre-clinical rodent studies demonstrated that [F-18]FGln demonstrated increased uptake in diseased pulmonary vessels and the right ventricle in a rodent model of PAH. The proposed research study will provide preliminary evidence of the potential to utilize [F-18]FGln as a non-invasive imaging biomarker of glutamine flux and metabolism across a range of PAH subjects.

The overall objective outlined in this study is to determine how pulmonary vascular remodeling in PAH at a cellular and pathological level is associated with changes in gas exchange physiology and hemodynamics (monitored with 129Xe MRI/MRS) and how these signals change with disease progression or treatment.

The prevalence of critical ab extrinsic compression of left main coronary artery (LMCA) is very high in patients with pulmonary arterial hypertension (PAH) symptomatic for angina (up to 40% according to a recent study of 121 patients with PAH). The element that most of all correlates with the degree of coronary stenosis is the diameter of the pulmonary artery (PA). In particular, a diameter ≥ 40 mm has a sensitivity of 83% and a specificity of 70% in patients with angina. Critical stenosis of LMCA is a risk factor for sudden death and in these condition percutaneous coronary angioplasty with stent implantation has proven to be a safe and effective long-term procedure. Preliminary data from a retrospective analysis of the registry of patients with PAH in Bologna (ARCA registry, 109/2016/U/Oss) highlights that even in PAH patients asymptomatic for angina, compression of LMCA can occur in up to 13% of patients and the main predictive parameter of compression was found to be a diameter ≥ 42 mm (with a sensitivity of 87% and a specificity of 77%). Performing a screening test by coronary-CT scan in all subjects suffering of PAH with a PA diameter ≥ 40 mm even if asymptomatic for angina could therefore help to identify patients with PAH at increased risk for sudden death at an early stage.

The purpose of this study is to learn what happens to macitentan and its active metabolite (aprocitentan) in the body of children aged between 1 month and 2 years.