Active clinical trials for "Familial Primary Pulmonary Hypertension"

The primary aim of this study was to evaluate exercise capacity, respiratory muscle strength, pulmonary function, dyspnea and physical activity levels in pediatric PAH patients and compare them with healthy controls. The secondary aim of the study was; To investigate the relationship of dyspnea with exercise capacity, respiratory muscle strength, respiratory functions, physical activity and blood count parameters in pediatric PAH patients.

The objectives of this study are to develop research methods and corresponding materials for a study of the effectiveness of sitaxentan sodium (Thelin) in "real-world" settings; to pilot test these methods at three study centers in Europe; and to refine and finalize research methods and corresponding materials for possible use in a follow-on, full-scale examination of the effectiveness of sitaxentan sodium (Thelin) in "real-world" settings.

This is a registry of patients diagnosed with pulmonary arterial hypertension, scheduled for treatment with Ventavis. Typical clinical measures and quality of life will be prospectively followed in 12 months period.

The purpose of the study is to find out how the blood flow changes in the lungs of people with pulmonary hypertension compared to healthy individuals without pulmonary hypertension. We would like to find out if there are differences in how blood flows when subjects are given a drug to dilate (widen) the arteries in their lungs and when they breathe extra oxygen. We will compare the results to when subjects don't receive any drug or extra oxygen. We hope that knowing about these differences will help us to better understand pulmonary hypertension and how to diagnose it earlier.

The purpose of this study is to demonstrate the safety and efficacy of inhaled Iloprost (Ventavis®) among adult Chinese patients with primary pulmonary hypertension, which is in compliance with Chinese SFDA regulation.

To investigate the long-term safety and effectiveness of Adcirca (tadalafil) in Pulmonary Arterial Hypertension (PAH) patients in the clinical practice -focused topics- To evaluate the incidence of adverse events for the patients with long-term use To evaluate the incidence of adverse events of decreased blood pressure, bleeding (including uterine hemorrhage), visual disturbance and sudden hearing loss.

Primary pulmonary hypertension (PPH) is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels for no apparent reason. The pulmonary artery is a blood vessel carrying oxygen-poor blood from the right ventricle (one of the heart's pumping chambers) to the lungs. In the lungs, the blood picks up oxygen, then flows to the heart's left side, where the left ventricle pumps it to the rest of the body through the aorta. In the United States, an estimated 500 to 1,000 new cases of primary pulmonary hypertension are diagnosed each year. The greatest number is reported in women between ages 20 and 40. However, men and women in all age ranges as well as very young children can develop PPH. Researchers have identified more than 40 BMPR2 mutations that can cause primary pulmonary hypertension. Many of these mutations introduce a stop signal that halts protein production prematurely, decreasing the amount of functional BMPR2 protein. Other mutations prevent the BMPR2 protein from reaching the cell surface, or alter its structure so it cannot form a complex with other proteins. It remains unclear how BMPR2 mutations cause primary pulmonary hypertension. Researchers suggest that a mutation in this gene prevents cell death or promotes cell proliferation, resulting in an overgrowth of cells in the blood vessels of the lungs. Cell overgrowth can narrow the diameter of the vessels, restricting blood flow and resulting in elevated blood pressure. However, it has not yet been reported regarding the genetic variants in Taiwan. Further details have been described in this project proposal.

Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by scarred blood clots in the blood vessels supplying the lungs. This in turn leads to failure of the right side of the heart. The reason why these scarred clots form is unknown. An operation to remove the scarred clots, known as pulmonary endarterectomy, is a potential cure. However, some patients have persistent obstructions within the blood vessels and heart failure even after surgery. It is thought that abnormal levels of proteins, found in the blood stream and responsible for inflammation and the development of new blood vessels may have role in causing the disease. In this study, these proteins were measured to assess whether they provide clues as to the cause of the disease and whether they could be used for the risk stratification of patients.

Pulmonary Arterial Hypertension is a progressive disease that has no cure. Patients die young and are limited in their daily activity. Current treatments only treat the symptoms of the disease rather than the underlying cause. At least 1 in 5 patients has a change in a gene called the bone morphogenetic type 2 protein (or BMPR2). Extensive evidence supports the concept of addressing the reduced levels of the BMPR2 protein to reverse disease. Through work already undertaken by this group, two potential therapies which increase BMPR2 have been identified for use in a future randomised control trial. In order for a clinical trial to be informative we need an accurate way of measuring the protein or the effects of the protein (known as a biomarker). This study will use blood samples taken from 17 patients and 30 healthy participants over various time-points (2-5 visits over 5 weeks for healthy controls; 2 visits, approximately four months apart for patients). Laboratory work will help identify the best biomarkers for subsequent therapy studies. By defining the best biomarkers we can speed up the drug development in this rare disease.

Cardiac magnetic resonance imaging has emerged as a potential valuable test for the early detection of Pulmonary Arterial Hypertension. A number of reports have provided some preliminary evidence that Pulmonary Artery (PA) stiffness may be accurately detected by imaging of the pulmonary artery in order to measure PA stiffness. In addition, cardiac MRI could play provide early and effective treatment for Pulmonary Arterial Hypertension (PAH).