Registration Study for Rare Type of Pulmonary Hypertension

Pulmonary Hypertension Clinical Trial

Official title:

Registration Study for Rare Type of Pulmonary Hypertension

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03169010
• Other study ID #: RarePH135
• Status: Recruiting
• Start date: May 6, 2017
• Est. completion date: December 31, 2040

Study information

• Verified date: September 2023
• Source: China National Center for Cardiovascular Diseases
• Contact: Xi-Qi XU, MD. PhD.
• Phone: +861088322267
• Email: xuxiqi0928[@]163.com
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical 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, progression, genetic and environmental contributions to disease.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 2000
• Est. completion date: December 31, 2040
• Est. primary completion date: December 31, 2040
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: N/A and older

Eligibility

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

Related Conditions & MeSH terms

• Hypertension
• Hypertension, Pulmonary
• Pulmonary Hypertension

Intervention

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.

Locations

Country	Name	City	State
China	Chinese Academy of Medical Sciences Fuwai Hospital and Peking Union Medical College Hospital	Beijing	Beijing

Sponsors (2)

Lead Sponsor	Collaborator
China National Center for Cardiovascular Diseases	Peking Union Medical College Hospital

Country where clinical trial is conducted

China, 

References & Publications (2)

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. — View Citation

Simonneau 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. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Survival Rate of Participants		up to 10 years, at 12 months interval
Primary	Lung transplantation		up to 10 years, at 12 months interval
Primary	Change in New York Heart Association (NYHA) functional class		up to 10 years, at 3 months interval
Primary	Change in 6 mint walk distance		up to 10 years, at 3 months interval
Secondary	Genetic alteration in participants with rare type of PH	To identify the major genetic alterations in participants with rare type of PH	Baseline
Secondary	Change in NT-proBNP		up to 10 years, at 3 months interval
Secondary	Change in hemodynamics		up to 10 years, at 6 months interval
Secondary	Change in cardiac function	Measured by Cardiac MRI	up to 10 years, at 3-6 months interval
Secondary	Pulmonary endarterectomy (PEA)	e.g. operated versus non-operated	up to 10 years, at 6 months interval
Secondary	Balloon pulmonary angioplasty (BPA)	e.g. BPA versus non-BPA	up to 10 years, at 6 months interval
Secondary	Medical treatment	e.g. mono- versus combination therapy	up to 10 years, at 6 months interval