Registration Study for Rare Type of Pulmonary Hypertension
- Conditions
- Pulmonary Hypertension
- Interventions
- Other: laboratory biomarker analysisGenetic: Genetic analysis
- Registration Number
- NCT03169010
- Lead Sponsor
- China National Center for Cardiovascular Diseases
- Brief Summary
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.
- Detailed Description
The main research contents of this registration study includes:
1. Build a baseline database of the rare type of pulmonary hypertension. Collect general information, on-set symptoms and time, laboratory examination, imaging results, right heart catheterization and treatment information.
2. Follow up recruited patients at regular intervals（6m\~1y). Collect information on change in patients condition, laboratory test and treatment.
3. Conduct genetic testing for gene mutation related or hereditary pulmonary hypertension. Link the clinical database to genetic database.
4. Establish bio-bank for serum/plasma, urine, stool, tissues or cells.
5. Establish prognostic study based on the clinical follow-up and genetic database.
6. Draw diagnostic and treatment algorithm for the rare type of pulmonary hypertension.
Controls subjects: blood sample and medical data collected once.
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- All
- Target Recruitment
- 2000
- Participant is willing and able to give informed consent for participation in the study.
- Patients diagnosed as idiopathic pulmonary artery hypertension, hereditary pulmonary artery hypertension, hereditary hemorrhagic telangiectasia associated pulmonary artery hypertension, pulmonary veno-occlusive disease, pulmonary capillary hemangiomatosis associated pulmonary artery hypertension, cavernous transformation of portal vein associated pulmonary artery hypertension, special type of congenital heart disease associated pulmonary artery hypertension, chronic thromboembolism pulmonary hypertension.
- All patients should have undergone right heart catheterization, diagnosed according to the guideline.
The participant may not enter the study if ANY of the following apply:
- Patients unwilling or unable to provide written consent for participation in the study.
- Not suffering from the rare type of pulmonary artery hypertension;
Inclusion criteria-Controls
- Participant is willing and able to give informed consent for participation in the study.
- Self-reported to be healthy
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description Pulmonary Takaysu Arteritis laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis. Pulmonary Veno-Occlusive Disease (PVOD) Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD. Idiopathic Pulmonary Artery Hypertension Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to idiopathic pulmonary artery hypertension (PAH). Hereditary PAH Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH. Pulmonary Veno-Occlusive Disease (PVOD) laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD. Cavernous Transformation of Portal Vein laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to cavernous transformation of portal vein associated PAH Cavernous Transformation of Portal Vein Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to cavernous transformation of portal vein associated PAH CTEPH Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH). Hereditary PAH laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH. Hereditary Hemorrhagic Telangiectasia laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary hemorrhagic telangiectasia associated PAH. Hereditary Hemorrhagic Telangiectasia Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary hemorrhagic telangiectasia associated PAH. Pulmonary Capillary Hemangiomatosis laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH Pulmonary Capillary Hemangiomatosis Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH Idiopathic Pulmonary Artery Hypertension laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to idiopathic pulmonary artery hypertension (PAH). Pulmonary Takaysu Arteritis Genetic analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis. CTEPH laboratory biomarker analysis Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH).
- Primary Outcome Measures
Name Time Method 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
- Secondary Outcome Measures
Name Time Method Change in NT-proBNP up to 10 years, at 3 months interval Pulmonary endarterectomy (PEA) up to 10 years, at 6 months interval e.g. operated versus non-operated
Genetic alteration in participants with rare type of PH Baseline To identify the major genetic alterations in participants with rare type of PH
Change in hemodynamics up to 10 years, at 6 months interval Change in cardiac function up to 10 years, at 3-6 months interval Measured by Cardiac MRI
Balloon pulmonary angioplasty (BPA) up to 10 years, at 6 months interval e.g. BPA versus non-BPA
Medical treatment up to 10 years, at 6 months interval e.g. mono- versus combination therapy
Trial Locations
- Locations (1)
Chinese Academy of Medical Sciences Fuwai Hospital and Peking Union Medical College Hospital
🇨🇳Beijing, Beijing, China