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| Name | Class |
|---|---|
| Peking Union Medical College Hospital | OTHER |
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The knowledge on the rare type of pulmonary hypertension which can not be explained by left heart disease, respiratory disease or congenital heart disease is very limited. Investigators aim to setup a national registration study for the rare type of pulmonary hypertension, to understand the natural history, survival, progression, genetic and environmental contributions to disease.
The main research contents of this registration study includes:
Controls subjects: blood sample and medical data collected once.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Idiopathic Pulmonary Artery Hypertension | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to idiopathic pulmonary artery hypertension (PAH). |
| |
| Hereditary PAH | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH. |
| |
| Hereditary Hemorrhagic Telangiectasia | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary hemorrhagic telangiectasia associated PAH. |
| |
| Pulmonary Veno-Occlusive Disease (PVOD) | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD. |
| |
| Pulmonary Capillary Hemangiomatosis | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH |
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| laboratory biomarker analysis | Other | Laboratory results will be analysed to identify disease related biomarkers. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Survival Rate of Participants | up to 10 years, at 12 months interval | |
| Lung transplantation | up to 10 years, at 12 months interval | |
| Change in New York Heart Association (NYHA) functional class | up to 10 years, at 3 months interval | |
| Change in 6 mint walk distance | up to 10 years, at 3 months interval |
| Measure | Description | Time Frame |
|---|---|---|
| Genetic alteration in participants with rare type of PH | To identify the major genetic alterations in participants with rare type of PH | Baseline |
| Change in NT-proBNP | up to 10 years, at 3 months interval |
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Inclusion Criteria:
Exclusion Criteria:
The participant may not enter the study if ANY of the following apply:
Inclusion criteria-Controls
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Rare type of pulmonary artery hypertension
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Xi-Qi XU, MD. PhD. | Contact | +861088322267 | xuxiqi0928@163.com | |
| Xin JIANG, MD. PhD. | Contact | +861088396016 | jxcs983@163.com |
| Name | Affiliation | Role |
|---|---|---|
| Zhi-Cheng JING, MD. PhD. | Chinese Academy of Medical Sciences, Fuwai Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Chinese Academy of Medical Sciences Fuwai Hospital and Peking Union Medical College Hospital | Recruiting | Beijing | Beijing Municipality | 100037 | China |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 24355643 | Background | Galie N, Corris PA, Frost A, Girgis RE, Granton J, Jing ZC, Klepetko W, McGoon MD, McLaughlin VV, Preston IR, Rubin LJ, Sandoval J, Seeger W, Keogh A. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D60-72. doi: 10.1016/j.jacc.2013.10.031. | |
| 19555858 | Background |
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Consent for sharing of non identifiable study data for regulatory authorities.
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| ID | Term |
|---|---|
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
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| ID | Term |
|---|---|
| D005820 | Genetic Testing |
| ID | Term |
|---|---|
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D008919 | Investigative Techniques |
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All subjects will have a sample of blood taken for Sanger or whole genome sequencing.
|
| Cavernous Transformation of Portal Vein | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to cavernous transformation of portal vein associated PAH |
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| CTEPH | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH). |
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| Pulmonary Takaysu Arteritis | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis. |
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| Genetic analysis | Genetic | Gene sequencing results will be analysed to identify disease related mutations. |
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| Change in hemodynamics | up to 10 years, at 6 months interval |
| Change in cardiac function | Measured by Cardiac MRI | up to 10 years, at 3-6 months interval |
| Pulmonary endarterectomy (PEA) | e.g. operated versus non-operated | up to 10 years, at 6 months interval |
| Balloon pulmonary angioplasty (BPA) | e.g. BPA versus non-BPA | up to 10 years, at 6 months interval |
| Medical treatment | e.g. mono- versus combination therapy | up to 10 years, at 6 months interval |
| Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, Elliott CG, Gaine SP, Gladwin MT, Jing ZC, Krowka MJ, Langleben D, Nakanishi N, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009 Jun 30;54(1 Suppl):S43-S54. doi: 10.1016/j.jacc.2009.04.012. |
| D002318 |
| Cardiovascular Diseases |
| D005821 | Genetic Techniques |
| D033142 | Genetic Services |
| D006296 | Health Services |
| D005159 | Health Care Facilities Workforce and Services |
| D003954 | Diagnostic Services |
| D011314 | Preventive Health Services |