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Registration Study for Rare Type of Pulmonary Hypertension

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ClinicalTrials.gov Identifier: NCT03169010
Recruitment Status : Recruiting
First Posted : May 30, 2017
Last Update Posted : September 11, 2017
Sponsor:
Information provided by (Responsible Party):
Zhi-Cheng JING, MD, 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.

Condition or disease Intervention/treatment
Pulmonary Hypertension Other: laboratory biomarker analysis Genetic: Genetic analysis

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.


Study Type : Observational [Patient Registry]
Estimated Enrollment : 2000 participants
Observational Model: Cohort
Time Perspective: Prospective
Target Follow-Up Duration: 5 Years
Official Title: Registration Study for Rare Type of Pulmonary Hypertension
Actual Study Start Date : May 6, 2017
Estimated Primary Completion Date : December 31, 2022
Estimated Study Completion Date : December 31, 2022

Resource links provided by the National Library of Medicine


Group/Cohort Intervention/treatment
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).
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

Hereditary PAH
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary PAH.
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

Hereditary Hemorrhagic Telangiectasia
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to hereditary hemorrhagic telangiectasia associated PAH.
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

Pulmonary Veno-Occlusive Disease (PVOD)
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to PVOD.
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

Pulmonary Capillary Hemangiomatosis
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to pulmonary capillary hemangiomatosis associated PAH
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

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
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.

CTEPH
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to chronic thromboembolism pulmonary hypertension (CTEPH).
Other: laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.

Genetic: Genetic analysis
Gene sequencing results will be analysed to identify disease related mutations.




Primary Outcome Measures :
  1. Survival Rate of Participants [ Time Frame: up to 5 years, at 12 months interval ]
    The percentage of participants who are alive


Secondary Outcome Measures :
  1. Genetic alteration in participants with rare type of PH [ Time Frame: Baseline ]
    To identify the major genetic alterations in participants with rare type of PH


Biospecimen Retention:   Samples With DNA
All subjects will have a sample of blood taken for Sanger or whole genome sequencing.


Information from the National Library of Medicine

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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
Sampling Method:   Non-Probability Sample
Study Population
Rare type of pulmonary artery hypertension
Criteria

Inclusion Criteria:

  • 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.

Exclusion Criteria:

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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03169010


Contacts
Contact: Xi-Qi XU, MD. PhD. +861088322267 xuxiqi0928@163.com
Contact: Xin JIANG, MD. PhD. +861088396016 jxcs983@163.com

Locations
China, Beijing
Chinese Academy of Medical Sciences Fuwai Hospital Recruiting
Beijing, Beijing, China, 100037
Contact: Xi-Qi XU, MD. PhD.    +861088322267    xuxiqi0928@163.com   
Contact: Xin JIANG, MD. PhD.    +861088396016    jxcs983@163.com   
Sponsors and Collaborators
China National Center for Cardiovascular Diseases
Investigators
Principal Investigator: Zhi-Cheng JING, MD. PhD. Chinese Academy of Medical Sciences, Fuwai Hospital

Publications:
Responsible Party: Zhi-Cheng JING, MD, Director of Thrombosis and Hemostasis Center of Chinese Academy of Medical Sciences Fuwai Hospital, China National Center for Cardiovascular Diseases
ClinicalTrials.gov Identifier: NCT03169010     History of Changes
Other Study ID Numbers: RarePH135
First Posted: May 30, 2017    Key Record Dates
Last Update Posted: September 11, 2017
Last Verified: September 2017
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: Undecided
Plan Description: Consent for sharing of non identifiable study data for regulatory authorities.

Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Zhi-Cheng JING, MD, China National Center for Cardiovascular Diseases:
registry
pulmonary hypertension
survival
biomarker
whole genome sequencing

Additional relevant MeSH terms:
Hypertension
Hypertension, Pulmonary
Vascular Diseases
Cardiovascular Diseases
Lung Diseases
Respiratory Tract Diseases