Pulmonary HypertensionNCT03169010
Registration Study for Rare Type of Pulmonary Hypertension
An observational study of Pulmonary Hypertension, run by China National Center for Cardiovascular Diseases at 1 site in China.
Trial 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… Read more about this study
- Who is it for?
- Any ageHealthy volunteers can take part
- Conditions the study is for
- Pulmonary Hypertension
- What is being tested?
- laboratory biomarker analysis and Genetic analysis
- How many people
- 2,000 participants
- Where
- 1 siteChina
- Study dates
- 6 May 2017to 31 Dec 2040
- How long will I take part?
- 20 Years
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages Any age
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
Study population: Rare type of pulmonary artery hypertension
Who may be able to join
- 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.
- Participant is willing and able to give informed consent for participation in the study.
- Self-reported to be healthy
Who cannot join
- 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;
Trial locations and contacts
1 site in 1 country. Contact the study team or a site near you to ask about taking part.
China1 site, 1 recruiting
- Recruiting
Chinese Academy of Medical Sciences Fuwai Hospital and Peking Union Medical College Hospital
Beijing, Beijing Municipality, 100037
Xi-Qi XU, MD. PhD., +861088322267, xuxiqi0928@163.com
Xin JIANG, MD. PhD., +861088396016, jxcs983@163.com
About this study
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.
Read the detailed description
The main research contents of this registration study includes:
- 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.
- Follow up recruited patients at regular intervals（6m~1y). Collect information on change in patients condition, laboratory test and treatment.
- Conduct genetic testing for gene mutation related or hereditary pulmonary hypertension. Link the clinical database to genetic database.
- Establish bio-bank for serum/plasma, urine, stool, tissues or cells.
- Establish prognostic study based on the clinical follow-up and genetic database.
- Draw diagnostic and treatment algorithm for the rare type of pulmonary hypertension.
Controls subjects: blood sample and medical data collected once.
- Conditions
- Pulmonary Hypertension
- Being tested
- laboratory biomarker analysis, Genetic analysis
- Keywords
- registry, pulmonary hypertension, survival, biomarker, whole genome sequencing
- Medical subject headings
- Hypertension, Pulmonary
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
laboratory biomarker analysisOther
Laboratory results will be analysed to identify disease related biomarkers.
Genetic analysisGenetic
Gene sequencing results will be analysed to identify disease related mutations.
| Study group | Role | What they receive |
|---|---|---|
| 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). Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| Hereditary PAH | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH. Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| Hereditary Hemorrhagic Telangiectasia | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary hemorrhagic telangiectasia associated PAH. Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| Pulmonary Veno-Occlusive Disease (PVOD) | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD. Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| Pulmonary Capillary Hemangiomatosis | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| 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 Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| CTEPH | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH). Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis | |
| Pulmonary Takaysu Arteritis | Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis. Receives: Other: laboratory biomarker analysis; Genetic: Genetic analysis |
What is being measured
The primary outcome is the main question the study is designed to answer.
Survival Rate of Participants
Measured: up to 10 years, at 12 months interval
Lung transplantation
Measured: up to 10 years, at 12 months interval
Change in New York Heart Association (NYHA) functional class
Measured: up to 10 years, at 3 months interval
Change in 6 mint walk distance
Measured: up to 10 years, at 3 months interval
Secondary outcomes (7)
Genetic alteration in participants with rare type of PH
To identify the major genetic alterations in participants with rare type of PH
Measured: Baseline
Change in NT-proBNP
Measured: up to 10 years, at 3 months interval
Change in hemodynamics
Measured: up to 10 years, at 6 months interval
Change in cardiac function
Measured by Cardiac MRI
Measured: up to 10 years, at 3-6 months interval
Pulmonary endarterectomy (PEA)
e.g. operated versus non-operated
Measured: up to 10 years, at 6 months interval
Balloon pulmonary angioplasty (BPA)
e.g. BPA versus non-BPA
Measured: up to 10 years, at 6 months interval
Medical treatment
e.g. mono- versus combination therapy
Measured: up to 10 years, at 6 months interval
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted30 May 2017
- Study start6 May 2017
- Primary completion31 Dec 2040 (estimated)
- Study completion31 Dec 2040 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (2)Papers the sponsor has linked to this study.
- Other trials for Pulmonary HypertensionStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 2,000 participants (estimated)
- Target duration
- 20 Years
Sponsor and investigators
- Lead sponsor
- China National Center for Cardiovascular Diseases
- Sponsor type
- Other government
- Collaborators
- Peking Union Medical College Hospital
- Responsible party
- Principal investigator, Zhi-Cheng JING, MD, China National Center for Cardiovascular Diseases
- Investigators
- Zhi-Cheng JING, MD. PhD., Principal investigator, Chinese Academy of Medical Sciences, Fuwai Hospital
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT03169010
- Sponsor's protocol ID
- RarePH135
- Status last verified
- Sep 2023
- First submitted
- 23 May 2017
- Last updated
- 2 Oct 2023
- Results posted
- No
- Data monitoring committee
- Yes
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- Undecided: Consent for sharing of non identifiable study data for regulatory authorities.
Publications
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.
PubMed 24355643Simonneau 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.
PubMed 19555858
Study at a glance
From the registry record, last updated by the sponsor on 2 Oct 2023. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Registration Study for Rare Type of Pulmonary Hypertension
- ClinicalTrials.gov ID
- NCT03169010
- Acronym / sponsor ID
- RarePH135
- Conditions
- Pulmonary Hypertension
- Keywords
- registry, pulmonary hypertension, survival, biomarker, whole genome sequencing
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 2,000 participants (estimated)
- Lead sponsor
- China National Center for Cardiovascular DiseasesOther government
- Collaborators
- Peking Union Medical College Hospital
- Study start
- 6 May 2017 (actual)
- Primary completion
- 31 Dec 2040 (estimated)
- Study completion
- 31 Dec 2040 (estimated)
- First posted
- 30 May 2017 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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:
- 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.
- Follow up recruited patients at regular intervals（6m~1y). Collect information on change in patients condition, laboratory test and treatment.
- Conduct genetic testing for gene mutation related or hereditary pulmonary hypertension. Link the clinical database to genetic database.
- Establish bio-bank for serum/plasma, urine, stool, tissues or cells.
- Establish prognostic study based on the clinical follow-up and genetic database.
- Draw diagnostic and treatment algorithm for the rare type of pulmonary hypertension.
Controls subjects: blood sample and medical data collected once.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Target follow-up
- 20 Years
- Number of arms
- 8
| Arm | Type | Receives |
|---|---|---|
| Idiopathic Pulmonary Artery Hypertension | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to idiopathic pulmonary artery hypertension (PAH). |
| Hereditary PAH | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH. |
| Hereditary Hemorrhagic Telangiectasia | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators 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) | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD. |
| Pulmonary Capillary Hemangiomatosis | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH |
| Cavernous Transformation of Portal Vein | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to cavernous transformation of portal vein associated PAH |
| CTEPH | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH). |
| Pulmonary Takaysu Arteritis | – | Other: laboratory biomarker analysis, Genetic: Genetic analysisInvestigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis. |
Who may qualify
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
- Study population
- Rare type of pulmonary artery hypertension
Key inclusion criteria (5 listed)
- 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.
- Participant is willing and able to give informed consent for participation in the study.
- Self-reported to be healthy
Key exclusion criteria (3 listed)
- Patients unwilling or unable to provide written consent for participation in the study.
- Not suffering from the rare type of pulmonary artery hypertension;
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| laboratory biomarker analysisLaboratory results will be analysed to identify disease related biomarkers. | Other | CTEPH, Cavernous Transformation of Portal Vein, Hereditary Hemorrhagic Telangiectasia, Hereditary PAH, Idiopathic Pulmonary Artery Hypertension, Pulmonary Capillary Hemangiomatosis, Pulmonary Takaysu Arteritis, Pulmonary Veno-Occlusive Disease (PVOD) |
| Genetic analysisGene sequencing results will be analysed to identify disease related mutations. | Genetic | CTEPH, Cavernous Transformation of Portal Vein, Hereditary Hemorrhagic Telangiectasia, Hereditary PAH, Idiopathic Pulmonary Artery Hypertension, Pulmonary Capillary Hemangiomatosis, Pulmonary Takaysu Arteritis, Pulmonary Veno-Occlusive Disease (PVOD) |
Primary outcome
Survival Rate of Participants
Time frame: up to 10 years, at 12 months interval
Lung transplantation
Time frame: up to 10 years, at 12 months interval
Change in New York Heart Association (NYHA) functional class
Time frame: up to 10 years, at 3 months interval
Change in 6 mint walk distance
Time frame: up to 10 years, at 3 months interval
7 secondary outcomes
Genetic alteration in participants with rare type of PH
Time frame: Baseline
Change in NT-proBNP
Time frame: up to 10 years, at 3 months interval
Change in hemodynamics
Time frame: up to 10 years, at 6 months interval
Change in cardiac function
Time frame: up to 10 years, at 3-6 months interval
Pulmonary endarterectomy (PEA)
Time frame: up to 10 years, at 6 months interval
Balloon pulmonary angioplasty (BPA)
Time frame: up to 10 years, at 6 months interval
Medical treatment
Time frame: up to 10 years, at 6 months interval
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 2 Oct 2023.
- Background: PubMed 24355643Galie 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.
- Background: PubMed 19555858Simonneau 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.
- Individual participant dataSharing planned: Undecided. Consent for sharing of non identifiable study data for regulatory authorities.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Zhi-Cheng JING, MD. PhD. principal investigator
Chinese Academy of Medical Sciences, Fuwai Hospital
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Pulmonary Hypertension, run by China National Center for Cardiovascular Diseases at 1 site in China.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 5 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 2 Oct 2023. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in China. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Xi-Qi XU, MD. PhD. (+861088322267, xuxiqi0928@163.com), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Survival Rate of Participants (time frame: up to 10 years, at 12 months interval). The study lists 4 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT03169010, which also lists 2 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 2 Oct 2023.