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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.
baseline include: An echocardiogram to assess the size, shape, pumping action and the extent of any damage to the heart.
Lung function tests which include blowing measurements to assess gas volumes within the lungs as well as assessment of how the lungs exchange gases.
Right heart catheterisation (RHC) to diagnose PAH Optional Cardiac Magnetic Resonance tests. 6 minute walk distance (6MWD). To measure exercise capacity Electrocardiogram (ECG), a test that measures the electrical activity of the heart Blood tests
main outcome measure include:
death heart/lung transplantation atrial septostomy hospitalization due to worsening of PAH start of new specific PAH treatment persistent decrease of >15% from baseline in 6MWD (or >30% compared with last study-related measurement) persistent worsening of World Health Organization (WHO) Functional Class (FC)
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| standard of care | Other | No intervention, this a patient registry and is purely an observational study |
| Measure | Description | Time Frame |
|---|---|---|
| survival | To recruit a cohort of CHD-PAH cases. define the death as the primary outcome | 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| clinical worsening | number of patients were dead, or having heart/lung transplantation, atrial septostomy, hospitalization due to worsening of PAH, starting of new specific PAH treatment, persistent decrease of >15% from baseline in 6MWD (or >30% compared with last study-related measurement), persistent worsening of WHO FC | 5 years |
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Inclusion Criteria:
-Clinical diagnosis of CHD-PAH (by RHC)
Exclusion Criteria:
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patients with diagnosis of CHD-PAH
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Zheng Xuan | Contact | +8618627885144 | soniazheng@outlook.com |
| Name | Affiliation | Role |
|---|---|---|
| Qiu Qiu | Wuhan Asian Heart Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Wuhan Asian Heart Hospital | Recruiting | Wuhan | China |
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| ID | Term |
|---|---|
| D000081029 | Pulmonary Arterial Hypertension |
| ID | Term |
|---|---|
| D006976 | Hypertension, Pulmonary |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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| ID | Term |
|---|---|
| D059039 | Standard of Care |
| ID | Term |
|---|---|
| D019984 | Quality Indicators, Health Care |
| D011787 | Quality of Health Care |
| D006298 | Health Services Administration |
| D017530 | Health Care Quality, Access, and Evaluation |
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