Pulmonary Hypertension Modality of Death and Validation of REVEAL Risk Score
Pulmonary Arterial HypertensionPulmonary Arterial Hypertension (PAH) is a chronic disease characterized by a progressive increase in pulmonary vascular resistance (PVR), which leads to right ventricular (RV) failure, and ultimately death. Different studies have outlined how various factors as vascular resistance, functional class, age, correlate with mortality. However, the modality of death and risk factors for mortality in patients with PAH are little known. For this purpose, more studies are necessary to analyze the risk factors related to modality of death in PAH.
Central China Congenital Heart Disease Associated Pulmonary Arterial Hypertension Cohort Study
Pulmonary Arterial HypertensionThe 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.
Imaging Control Study, 3D Echo, MR and RHC.
Pulmonary Arterial Hypertension WHO Group IThe 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.
3D Imaging: Prognostic Role in Pulmonary Arterial Hypertension
Pulmonary Arterial HypertensionPulmonary 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.
Observation Study With Implantable Medication Pump for Intravenous Treprostinil Therapy in Patients...
Pulmonary Arterial HypertensionPAHThe 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).
Persistent Pulmonary Artery Hypertension After Valve Replacement
Persistent Severe PH After Valve TherapyBackground: Persistent severe pulmonary hypertension (PH) after mechanical valve replacement is a frequent finding in patients with severe valve-diseases. The reasons to develop PH are multifactorial and the prevalence of severe PH significantly worsens the patients´ outcome. Aims: (i) To define the prevalence of PH in patients after valve replacement and (ii) to identify factors predicting persistent PH. Methods: All patients which underwent valve replacement at our institution between the years 2008 -2010 will be screened retrospectively. Those patients with pre- procedural proven PH by means of right-heart catheterization will receive prospective follow-up with echocardiography. Diagnosis of persistent PH will be confirmed with right heart catheterization.
The PAH Disability and Bothersomeness Questionnaire
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.
Is There Intravascular Hemolysis in Patients With Pulmonary Hypertension?
Pulmonary Artery HypertensionPatients with hemolytic disorders (e.g. sickle cell anemia or thalassemia) are known to develop pulmonary hypertension. Hemolysis is where red blood cells are destroyed and their contents released into the circulation. It is thought that these red-cell contents cause constriction and thrombosis of the blood vessels in the lungs. Conversely, it is possible that patients with pulmonary hypertension have hemolysis. In this study we will be drawing blood from a range of patients and normal controls for a panel of blood tests related to hemolysis.
Volatile Organic Compounds (VOCs) as a Biomarker in Immune-mediated Pulmonary Arterial Hypertension...
Pulmonary Arterial HypertensionSystemic Sclerosis1 moreAim: 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.
3D Echo Evaluation of the Ventricles in Sclero Associated Severe PAH
Pulmonary Arterial HypertensionThe aim of the study is to document the left and right ventricular function in patients with sclerodermia associated pulmonal arterial hypertension via 3D echocardiography. The results of this study should help to generate hypothesis for further future studies. The primary hypothesis is that the increased use of modern image guided methods could provide essential aspects for the follow up.