Registration Study for Rare Type of Pulmonary Hypertension

September 28, 2023 updated by: Zhi-Cheng JING, MD, China National Center for Cardiovascular Diseases
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.

Study Overview

Status

Recruiting

Conditions

Intervention / Treatment

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

Enrollment (Estimated)

2000

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

  • Name: Xin JIANG, MD. PhD.
  • Phone Number: +861088396016
  • Email: jxcs983@163.com

Study Locations

  • China
    • Beijing
      • Beijing, Beijing, China, 100037
        • Recruiting
        • Chinese Academy of Medical Sciences Fuwai Hospital and Peking Union Medical College Hospital
        • Contact:
        • Contact:

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult
  • Older Adult

Accepts Healthy Volunteers

Yes

Sampling Method

Non-Probability Sample

Study Population

Rare type of pulmonary artery hypertension

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

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.
Pulmonary Takaysu Arteritis
Investigators will conduct laboratory biomarker analysis and genetic analysis to identify pathogenesis or factors related to Pulmonary Takaysu Arteritis.
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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
Survival Rate of Participants
Time Frame: up to 10 years, at 12 months interval
up to 10 years, at 12 months interval
Lung transplantation
Time Frame: up to 10 years, at 12 months interval
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
up to 10 years, at 3 months interval
Change in 6 mint walk distance
Time Frame: up to 10 years, at 3 months interval
up to 10 years, at 3 months interval

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
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
Baseline
Change in NT-proBNP
Time Frame: up to 10 years, at 3 months interval
up to 10 years, at 3 months interval
Change in hemodynamics
Time Frame: up to 10 years, at 6 months interval
up to 10 years, at 6 months interval
Change in cardiac function
Time Frame: up to 10 years, at 3-6 months interval
Measured by Cardiac MRI
up to 10 years, at 3-6 months interval
Pulmonary endarterectomy (PEA)
Time Frame: up to 10 years, at 6 months interval
e.g. operated versus non-operated
up to 10 years, at 6 months interval
Balloon pulmonary angioplasty (BPA)
Time Frame: up to 10 years, at 6 months interval
e.g. BPA versus non-BPA
up to 10 years, at 6 months interval
Medical treatment
Time Frame: up to 10 years, at 6 months interval
e.g. mono- versus combination therapy
up to 10 years, at 6 months interval

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

China National Center for Cardiovascular Diseases

Collaborators

Peking Union Medical College Hospital

Investigators

  • Principal Investigator: Zhi-Cheng JING, MD. PhD., Chinese Academy of Medical Sciences, Fuwai Hospital

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

May 6, 2017

Primary Completion (Estimated)

December 31, 2040

Study Completion (Estimated)

December 31, 2040

Study Registration Dates

First Submitted

May 23, 2017

First Submitted That Met QC Criteria

May 25, 2017

First Posted (Actual)

May 30, 2017

Study Record Updates

Last Update Posted (Actual)

October 2, 2023

Last Update Submitted That Met QC Criteria

September 28, 2023

Last Verified

September 1, 2023

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • RarePH135

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

IPD Plan Description

Consent for sharing of non identifiable study data for regulatory authorities.

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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