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NCT Number: NCT03169010

Registration Study for Rare Type of Pulmonary Hypertension

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.

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Key information

Conditions

Pulmonary Hypertension Cardiovascular Diseases Hypertension Hypertension, Pulmonary Lung Diseases Respiratory Tract Diseases Vascular Diseases

Sex eligibility

All sexes

Study type

Observational

Primary location

About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

laboratory biomarker analysis

Other

Laboratory results will be analysed to identify disease related biomarkers.

Genetic analysis

Genetic

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

Primary outcomes

  1. Survival Rate of Participants

    Time frame: up to 10 years, at 12 months interval

  2. Lung transplantation

    Time frame: up to 10 years, at 12 months interval

  3. Change in New York Heart Association (NYHA) functional class

    Time frame: up to 10 years, at 3 months interval

  4. Change in 6 mint walk distance

    Time frame: up to 10 years, at 3 months interval

Secondary outcomes

  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

  2. Change in NT-proBNP

    Time frame: up to 10 years, at 3 months interval

  3. Change in hemodynamics

    Time frame: up to 10 years, at 6 months interval

  4. Change in cardiac function

    Time frame: up to 10 years, at 3-6 months interval

    Measured by Cardiac MRI

  5. Pulmonary endarterectomy (PEA)

    Time frame: up to 10 years, at 6 months interval

    e.g. operated versus non-operated

  6. Balloon pulmonary angioplasty (BPA)

    Time frame: up to 10 years, at 6 months interval

    e.g. BPA versus non-BPA

  7. Medical treatment

    Time frame: up to 10 years, at 6 months interval

    e.g. mono- versus combination therapy

Study contacts

Contact information is provided by the study sponsor or research team.

Xi-Qi XU, MD. PhD.

CONTACT

[email protected]

+861088322267

Xin JIANG, MD. PhD.

CONTACT

[email protected]

+861088396016

Sponsors and collaborators

Lead sponsor

China National Center for Cardiovascular Diseases

Other Gov

Collaborators

  • Peking Union Medical College Hospital

Registry information

Important dates

Study start
2017
Primary completion
2040
Study completion
2040
First posted
May 30, 2017
Registry last updated
Oct 2, 2023

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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