Gene Mutations in Secondary Pulmonary Hypertension
Summary
As a pulmonary hypertension center, we have created a database that prospectively tracks patients with different forms of secondary pulmonary hypertension. Records include genetic analysis, and clinical and hemodynamic profiles.
Study Design
Observational Model: Defined Population, Time Perspective: Longitudinal, Time Perspective: Prospective
Conditions
Chronic Thromboembolic Pulmonary Hypertension
Location
Medical University of Vienna
Vienna
Austria
1090
Status
Recruiting
Source
Medical University of Vienna
Results (where available)
Links
- Source: http://clinicaltrials.gov/show/NCT00348803
- Information obtained from ClinicalTrials.gov on July 15, 2010
Medical and Biotech [MESH] Definitions
Pulmonary Heart Disease
Hypertrophy and dilation of the RIGHT VENTRICLE of the heart that is caused by PULMONARY HYPERTENSION. This condition is often associated with pulmonary parenchymal or vascular diseases, such as CHRONIC OBSTRUCTIVE PULMONARY DISEASE and PULMONARY EMBOLISM.
Pulmonary Disease, Chronic Obstructive
A disease of chronic diffuse irreversible airflow obstruction. Subcategories of COPD include CHRONIC BRONCHITIS and PULMONARY EMPHYSEMA.
Epoprostenol
A prostaglandin that is a powerful vasodilator and inhibits platelet aggregation. It is biosynthesized enzymatically from PROSTAGLANDIN ENDOPEROXIDES in human vascular tissue. The sodium salt has been also used to treat primary pulmonary hypertension (HYPERTENSION, PULMONARY).
Hepatopulmonary Syndrome
A syndrome characterized by the clinical triad of advanced chronic liver disease, pulmonary vascular dilatations, and reduced arterial oxygenation (HYPOXEMIA) in the absence of intrinsic cardiopulmonary disease. This syndrome is common in the patients with LIVER CIRRHOSIS or portal hypertension (HYPERTENSION, PORTAL).
Poems Syndrome
A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)
Clinical Trials
Bosentan Effects in Inoperable Forms of Chronic Thromboembolic Pulmonary Hypertension
The present trial investigates a possible use of oral bosentan, which is currently approved for the treatment of symptoms of pulmonary arterial hypertension (PAH), to patients suffering fr...
In order to improve effectiveness of the surgical operation thanks to a reduction in the rate of failure, this study will allow us to identify predictive angiographic and CT finding of sur...
Bosentan in Patients With Inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
The present trial investigates the long-term safety, tolerability and efficacy of bosentan in patients with inoperable CTEPH.
A Study to Evaluate Efficacy and Safety of Oral BAY63-2521 in Patients With CTEPH.
The aim of the study is to assess the efficacy and safety of different doses of BAY63-2521, given orally for 16 weeks, in patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH...
Sildenafil is a phosphodiesterase-5 inhibitor that has been approved for the treatment of pulmonary arterial hypertension with orphan drug designation. Sildenafil modulates the nitric oxid...
PubMed Articles
Chronic Thromboembolic Pulmonary Hypertension.
Chronic thromboembolic pulmonary hypertension can develop after acute pulmonary embolus but is often overlooked until pulmonary hypertension has led to dyspnea and right ventricular dysfunction. This...
Chronic thromboembolic pulmonary hypertension (CTEPH) results from chronic thrombotic occlusion of the pulmonary arterial circulation and may be potentially cured by pulmonary thromboendarterectomy. E...
Chronic thromboembolic pulmonary hypertension in pediatric patients.
OBJECTIVE: Chronic thromboembolic pulmonary hypertension is a rare form of pulmonary hypertension that can lead to progressive right heart failure and death. Pulmonary thromboendarterectomy surgery is...
Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with abnormal left ventricular (LV) filling hemodynamics (mitral E/A
