Clinical trial
Registration Study for Rare Type of Pulmonary Hypertension
Name
RarePH135
Description
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.
Trial arms
Trial start
2017-05-06
Estimated PCD
2040-12-31
Trial end
2040-12-31
Status
Recruiting
Treatment
laboratory biomarker analysis
Laboratory results will be analysed to identify disease related biomarkers.
Arms:
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 analysis
Gene sequencing results will be analysed to identify disease related mutations.
Arms:
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)
Size
2000
Primary endpoint
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
Eligibility 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
Protocol
{'studyType': 'OBSERVATIONAL', 'patientRegistry': True, 'targetDuration': '20 Years', 'designInfo': {'observationalModel': 'COHORT', 'timePerspective': 'PROSPECTIVE'}, 'bioSpec': {'retention': 'SAMPLES_WITH_DNA', 'description': 'All subjects will have a sample of blood taken for Sanger or whole genome sequencing.'}, 'enrollmentInfo': {'count': 2000, 'type': 'ESTIMATED'}}
Updated at
2023-10-02
1 organization
1 product
1 indication
Product
Genetic analysisIndication
Pulmonary Hypertension