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 investigate pulmonary hemodynamics at rest and during exercise in
patients before and after kidney transplantation.
Pulmonary arterial hypertension is a disease characterised by pathological changes in the
pulmonary arteries leading to a progressive increase in pulmonary vascular resistance and
pulmonary artery pressure. Right ventricular failure is the main cause of death in patients
with pulmonary arterial hypertension, and the ability of the right ventricle to adapt to the
progressive increase in pulmonary vascular resistance associated with changes to the
pulmonary vasculature in pulmonary arterial hypertension is the main determinant of a
patient's functional capacity and survival.Mesenchymal stem cells (MSCs)are a subset of adult
Chronic thromboembolic pulmonary hypertension (CTEPH) is the fourth group of pulmonary
hypertension (PH) according to clinical classification. Pulmonary endarterectomy（PEA) is the
preferred treatment for patients with CTEPH, however, PEA has its limitations, it only
applies to the thrombi in the main, lobar, or segmental pulmonary arteries for patients with
CTEPH, and the postoperative residual PH is found to be of high percentage. With the
development of interventional techniques, balloon pulmonary angioplasty (BPA) has been used
to treat chronic thrombotic pulmonary hypertension (CTEPH) with favorable results and has
Combination therapy in pulmonary arterial hypertension (PAH) has been the subject of active
investigation for more than a decade, with the benefit of targeting different pathways known
to be involved in the pathogenesis of the disease. Adherence to prescribed therapy has an
impact on clinical outcomes. Reducing the pill/tablet count and frequency has a major impact
on patients' adherence to therapies and therefore the observed clinical outcomes. One way to
simplify treatment is to use fixed-dose combination (FDC) products that combine multiple
treatments targeting different pathways into a single tablet.
This study aims to...
Though graded balloon-atrial-septostomy (BAS) has been accepted as an effective palliative
therapy for severe pulmonary arterial hypertension, spontaneous closure of septostomy is not
uncommon. Radiofrequency-catheter-ablation (RFA), which has the potential to cause
irreversible damage around the rim of created inter-atrial communication, might contribute to
prevent the spontaneous closure. In patients with severe pulmonary arterial hypertension, the
combined use of RFA and BAS (CURB) is investigated to create a stable inter-atrial
In view of the manifold options for mono- and combination therapy that have now emerged for
patients with pulmonary (arterial) hypertension (PH/PAH), controlled clinical trials can only
provide part of the information needed for optimal management. In order to gather adequate
data on PAH/PH treatment in routine clinical care, the ongoing COMPERA registry prospectively
documents consecutive patients with newly initiated treatment of PAH/PAH since May 2007. The
internet-based registry fulfills high quality standards through several measures (planned
minimum centre contribution of at least 10 patients per year, automated plausibility checks
To address the knowledge gap that exists among providers resulting in underdiagnosis of
chronic thromboembolic pulmonary hypertension (CTEPH), the investigators have devised this
400-patient single-center Quality Improvement Initiative in the form of a randomized
controlled trial of an EPIC Best Practice Advisory (BPA) on-screen alert versus no
notification to increase echocardiographic screening for CTEPH and the diagnosis of CTEPH in
patients with prior pulmonary embolism (PE) and symptoms/signs suggestive of pulmonary
hypertension or recent pulmonary testing suggesting unexplained respiratory symptoms at 3
The pathophysiological processes involved in COVID-19 pneumonia are not fully understood.
Specific alterations of the airways, lung parenchyma and pulmonary vascular tree could
explain a severe ventilation/perfusion heterogeneity resulting in severe hypoxemia during the
active phase of the disease. Additional skeletal muscle impairment related to systemic
inflammation may also explain persisting symptoms in the follow-up phase. The first aim of
the present project is to explore these different processes by evaluating the impact of the
COVID-19 pneumonia on exercise capacity, pulmonary function and perfusion by a physiological
Pulmonary hypertension (PH) is defined as a pulmonary arterial mean pressure (meanPAP) ≥ 25
mmHg measured in the right heart catheterization.
There are different forms of PH defined in the classification of Dana Point 2008.
PH is diagnosed with right heart catheterization but there are other non invasive methods
which can be used for screening like the echocardiography, stress echocardiography and cardio
pulmonary exercise testing. In the diagnosis process and in the follow up of PH patients
biomarkers like NTproBNP are helpful. There are no specific biomarkers for the disease which
can make the diagnosis process easier and...