ADAMTSL4 in Idiopathic Pulmonary Hypertension and CTEPH

Study Overview
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Brief Summary
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ADAMTSL4 (A disintegrin and metalloproteinase with thrombospondin motifs like) is a member of the ADAMTSL family. ADAMTSL4 is one of the family of 7 ADAMTS-Like proteins, themselves part of the Thrombospondin type 1 repeat (TSR) superfamily of proteins. These 7 proteins are divided into two distinct clades, of which ADAMTSL4 and ADAMTS-Like 6 form part of one clade, differing from ADAMTS-Like 1, ADAMTS-Like 3 and ADAMTS-Like 7 by lacking immunoglobulin repeat regions. ADAMTSL4 is widely expressed in brain, colon, heart, kidney, liver, lung, pancreas and spleen, yet, its precise role is unclear. in addition, ADAMTSL4 protein high expression to the medial layer of the arterial wall, and medial vascular smooth muscle cells specifically.

Detailed Description
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The objectives of the present study aimed to confirm ADAMTSL4 levels in the proteomics between healthy control population and patients with IPAH，to analyze the expression levels of ADAMTSL4 between patients with IPAH and CTEPH, and to investigate whether serum levels of ADAMTSL4 might be used as biomarkers of disease severity in these patient populations.

Official Title
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ADAMTSL4 in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Conditions
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Pulmonary Hypertension

Intervention / Treatment
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* Other: drugs or BPA


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: - Healthy people，patients with IPAH，patients with CTEPH Exclusion Criteria: - tumour，infection，Pregnancy, suckling period， Mental disorders， Other types of PAH except CTEPH and IPAH

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| control group<br>healthy people as control group | |
| IPAH group<br>patients with IPAH as IPAH group | Other: drugs or BPA<br>* PAH target drugs，for example， Tadanafiland, Maxitensin, Prostacyclins, riociguat and etc. CTEPH treat with balloon pulmonary angioplasty or riociguat<br>|
| CTEPH group<br>patients with CTEPH as CTEPH group | Other: drugs or BPA<br>* PAH target drugs，for example， Tadanafiland, Maxitensin, Prostacyclins, riociguat and etc. CTEPH treat with balloon pulmonary angioplasty or riociguat<br>|
| group D<br>Including CTEPH group and IPAH group | Other: drugs or BPA<br>* PAH target drugs，for example， Tadanafiland, Maxitensin, Prostacyclins, riociguat and etc. CTEPH treat with balloon pulmonary angioplasty or riociguat<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Association of clinic index and ADAMTSL4 expression level | The expression and its clinical relevance of ADAMTSL4 expression level in plasma or pulmonary tissue in patients with IPAH and CTEPH | 2018-2022 |