Riociguat in Patients With Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
HypertensionPulmonaryThe aim of the study is to assess safety, tolerability and clinical effects of different doses of riociguat in patients with inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and who are not satisfactorily treated and cannot participate in any other CTEPH trial. In the US the study runs as an Expanded Access program under 21 CFR 312.320.
Study on the Clinical Course Of Pulmonary Embolism
Cardiovascular DiseasesVenous Thromboembolism1 moreThe course of both pulmonary embolism (PE) and one of its more relevant late complications, i.e. chronic thromboembolic pulmonary hypertension (CTEPH) is still substantially unknown. Recent evidence has shown that the incidence of CTEPH is higher than previously believed, but this has not been confirmed by other studies. A clear link between PE and CTEPH has been questioned by some experts. A great number of patients affected by PE persistently have residual chronic thromboembolic material the meaning of which is a matter of debate. The evidence sustaining a link between chronic residual PE and subsequent PE recurrences or CTEPH is insufficient. Thus, a nationwide, multicentre, prospective cohort study was designed with the following aims: to ascertain the incidence of symptomatic CTEPH after a first episode of acute PE; to ascertain the incidence of venous thromboembolic (VTE) recurrences after a first episode of acute PE; to evaluate whether a relation exists between chronic residual PE and CTEPH to evaluate whether a relation exists between chronic residual PE and VTE recurrences; to evaluate whether a relation exists between persistent right ventricular dysfunction and CTEPH; to evaluate whether a relation exists between persistent right ventricular dysfunction and PE recurrences. For each enrolling centre, consecutive outpatients or inpatients with an objectively diagnosed first acute PE episode are considered eligible.
NIMS- New Implantable Pump in Pulmonary Arterial Hypertension, a Safety Follow up
Pulmonary Hypertension Group 1 and 4 According to Nice ClassificationThis observational study aims to chart patients which get their prostacyclin treatment via the subcutaneous LenusPro pump. How the surgical procedure is performed and the clinical follow-up of these patients. And by this also charting complications that might occur in relation to treatment. Participating centres are located in Denmark, Norway and Sweden.
Non-invasive Assessment of Pulmonary Artery Pressure
Pulmonary HypertensionThe assessment of pulmonary artery pressure (PAP) and parameters describing right ventricular function stand in the focus of the diagnosis and clinical management of pulmonary hypertension (PH). Right heart catheterization (RHC) is the gold standard method to measure PAP and to provide hemodynamic information on right ventricular function. However, due to its invasive nature, RHC is not optimal for screening and for close monitoring of the disease. Therefore, the development of non-invasive methods providing reliable PAP measurements and right ventricular functional parameters would be of major benefit. Today, the most often used comprehensive non-invasive method for these purposes is echocardiography. However, the method has limitations; in many cases PAP is significantly under- or overestimated - especially in subjects with co-existing pulmonary diseases. Regarding right ventricular function, although novel echocardiography parameters appear to be promising, they have not yet been evaluated in all forms of PH. Another emerging non-invasive method is cardiac magnetic resonance imaging (MRI). MRI is considered to be as gold standard for the non-invasive assessment of right ventricular function. In addition, our group showed that with a special approach ("vortex method"), MRI enables the determination of PAP with physiologic accuracy, but the method has not yet been validated systematically in different forms of PH. All patients undergoing right heart catheterization in our clinic are candidates for the study. Excluded will be patients not eligible for MRI or declining to take part in the study. MRI and Echocardiography will be performed within two weeks of the RHC. Hypothesis: MRI is superior to echocardiography to non-invasively determine mean PAP in a broad collective of patients with PH of diverse ethology. MRI derived right ventricular functional parameters correlate better to invasive measurements and to established prognostic parameters than echocardiography derived right ventricular functional parameters. Novel right ventricular tissue Doppler parameters add substantially to "classical" echocardiography parameters to describe right ventricular function.
CTEPH DIAGNOSIS Europe - MRI
Pulmonary HypertensionPhase III diagnostic trial to demonstrate that functional lung MRI can replace VQ-SPECT in a diagnostic strategy for patients with suspected CTEPH where positive findings are verified with catheter pulmonary angiography (CPA), or computed tomography pulmonary angiography (CTPA)
Idiopathic Pre-capillary Pulmonary Hypertension in ESKD Patients
Pulmonary HypertensionThis study investigates the prevalence and prognosis of idiopathic pre-capillary pulmonary hypertension (PH) in patients with end-stage kidney disease (ESKD).
HYPID (Pulmonary Hypertension in Interstitial Lung Disease) EXTENSION
Pulmonary Arterial HypertensionDiffuse Interstitial Lung DiseaseHYPID-2 study is an extension of HYPID study (NCT01443598) : HYPID-2 is also an observational and prospective study of patients with interstitial lung disease and pre capillary hypertension diagnosed by right heart sided catheterization. It concerns only incident patients (i.e patients included within 6 months after PH diagnosis) whereas HYPID concerned prevalent and incident cases. The primary aim is the same than HYPID : identify prognostic factors
Examination of Ventavis (Iloprost) Inhalation Behavior Using the I-Neb AAD System in Patients With...
HypertensionPulmonaryThe aim of this study is to examine inhalation behavior in patients enrolled in the German Ventavis patient support program Ventaplus when these patients are switched from Ventavis (Iloprost) 10 μg/mL to Ventavis (Iloprost) 20 μg/mL formulation.
Sleep Related Breathing Disturbances and High Altitude Pulmonary Hypertension in Kyrgyz Highlanders...
High Altitude Pulmonary HypertensionSleep ApneaHigh altitude pulmonary hypertension, a form of altitude illness that occurs in long-term residents at altitudes >2500 m, is characterized by dyspnea, hypoxemia, impaired exercise performance and hypertension in the pulmonary circulation. Whether sleep related breathing disturbances, common causes of nocturnal hypoxemia in lowlanders, are also prevalent in highlanders and promote pulmonary hypertension in highlanders is unknown. Therefore, the current study will investigate whether highlanders with high altitude pulmonary hypertension have a greater prevalence of sleep apnea than healthy highlanders and lowlanders.
Heart Disease in Sickle Cell Anemia
Sickle Cell AnemiaPulmonary HypertensionThis study will explore what may cause people with sickle cell anemia to have heart problems and an increased risk of sudden death. People 18 years of age and older with sickle cell anemia may be eligible for this study. Candidates are screened with a medical history and physical examination, electrocardiogram (EKG), echocardiogram (heart ultrasound), and blood tests. Participants undergo the following tests and procedures: Holter monitoring: The patient wears a small, battery-operated device to record heart rate and rhythm over 24 to 48 hours. QRST surface mapping: An EKG using 64 electrodes is done at rest and during exercise to provide a detailed look at the heart and its conduction system. Chest x-rays are taken to examine the lungs. Bicycle exercise echocardiography test: Blood pressure, pulse, heart rhythm and oxygen use are monitored while the patient exercises on a stationary bicycle. Ultrasound pictures are also obtained during the exercise. Echocardiogram: A heart ultrasound is done to check how well the heart is pumping blood. Pulmonary artery catheterization: A catheter (plastic tube) is inserted into a vein and advanced to the chambers of the heart, through the heart valve and into the lung artery. The pressures in the heart and lung blood vessels are measured while the patient is resting and during exercise, with the bed tilted up and down, and after giving 500 mls of fluid into a vein. Blood tests are done to measure a hormone called brain natriuretic peptide, which can increase with the development of heart failure, and nitrite, a substance that can affect blood vessel dilation. Some blood is stored to test for inflammatory markers and for possible future gene and protein analysis. Cardiac magnetic resonance imaging (cMRI): The patient lies in a donut-shaped magnet while pictures of the heart are obtained using a magnetic field and radio waves. Earplugs are worn to muffle the loud sounds that occur with electrical switching of the magnetic fields. A contrast agent called gadolinium may be injected to enhance the quality of the images. Invasive electrocardiographic (reveal) monitoring: This procedure permits study of the heart rhythms over a long time period. A small device is placed just under the skin on the left side of the chest. It can be left in for up to 14 months to monitor the heartbeat continuously during this time.