Active clinical trials for "Familial Primary Pulmonary Hypertension"

The investigators intend to explore the characteristics and outcomes of pregnant women with PAH during different pregnancies and to provide applicable evidence for clinical practice.

The study will recruit and follow up patients for five years patients newly diagnosed with congenital heart disease associated pulmonary arterial hypertension(CHD-PAH) from the investigator's hospital. The main aim of the study is to describe the aetiology, natural history and management practices of CHD-PAH in central China.

The aim of the study is to intensify the follow up of patients with pulmonary arterial hypertension via modern imaging guided methods in due consideration of the possibilities of three-dimensional echocardiography in order to optimize their specific therapy. The hypothesis is that the increased use of modern imaging guided tools is essential for the follow up.

Pulmonary hypertension is a rare and severe disease, affecting a young population. Survival is very poor and has been closely related to right ventricular dysfunction. Current prognostic equations rely mostly on right heart catheterization data. The identification of simple echocardiographic prognostic factor is urgently needed. It could help identifying with a non invasive method, high risk patients who could benefit from an intensive specific therapy. 3D right ventricular imaging is a new echocardiographic tool which provides RV volumic analysis, RV ejection fraction, overcoming the classical limits of 2D ultrasound. The aim of this study is to validate a new software for 3D analysis of the right ventricle and assess its prognostic role in pulmonary hypertension. To do so, the investigators will realize a prospective monocentric longitudinal cohort study, including 100 pulmonary hypertension patients. Echocardiographic data will be collected at baseline and after 6 months.

The study is designed as a non-interventional, multicenter, prospective single-arm study to observe the therapy with REMODULIN (Treprostinil) applicated by an implantable pump in patients with pulmonary arterial hypertension (PAH).

To develop a patient-reported questionnaire to investigate the impact of PAH (pulmonary arterial hypertension) on patients' daily lives in terms of bothersomeness and disability.

Aim: to investigate the role of inflammation and auto-immunity in pulmonary arterial hypertension by using the profile of volatile organic compounds. Hypothesis: first, the investigators hypothesize that at time of diagnosis the VOC profiles will discriminate patients with PAH-CTD and idiopathic PAH (IPAH) from patients with systemic sclerosis or systemic lupus erythematosus (CTD) without PAH, supporting the contention that there is a overlapping inflammatory and auto-immune pathway in PAH. During follow-up, the investigators will measure the VOC profiles of patients in all three groups who will be treated according standard clinical care. The hypothesis is that VOC profiles are affected by therapy.

The purpose of this study is to evaluate the role of stress echocardiography, compared to standard echocardiography in the early identification of pulmonary arterial hypertension in systemic sclerosis. To evaluate the role of BNP in this setting.To analyze data recorded with respect to the parameters commonly used for SSc evaluation (eg thorax HRCT, pulmonary function tests + DLCO, nailfold capillaroscopy, etc); these parameters are available starting for 1999.