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Pulmonary arterial hypertension (PAH) is a rare and incurable disease affecting people of all ages. It is characterized by obstructive remodeling of the small pulmonary arteries, responsible for an increase in pulmonary arterial pressure, leading to right heart failure and death in the absence of treatment. PAH can be associated with a variety of diseases, but around half of all PAH cases are idiopathic or hereditary, and may develop on predisposed terrain following a "second hit", as suggested by the identification of PAH cases associated with the use of anorectic drugs, methamphetamine and occupational exposure to organic solvents. No study has systematically analyzed the exposome of patients with PAH, combining environmental and occupational exposures as well as drugs and medications.
The exposome of patients with PAH without associated causes will be compared with that of patients with another form of pulmonary hypertension (PH), linked to thromboembolic risk factors: chronic thromboembolic PH (CTEPH), which will constitute the control group.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| PAH group (cases) | Patients with PAH without associated pathology: idiopathic or heritable, or with features of venous/capillary involvement, according to the clinical classification of PH |
| |
| CTEPH group (controls) | Patients with chronic thromboembolic PH (CTEPH, class 4), according to the clinical classification of PH |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Questionnaires | Other | Patients will complete questionnaires on professional exposures with the help of a trained professional (estimated time: 30 minutes), followed by self-questionnaires on indoor and outdoor pollution, medications, drugs and socio-economic variables (estimated time: 30 minutes). |
| Measure | Description | Time Frame |
|---|---|---|
| To compare the concentration of 180 environmental toxins of interest in the hair and urine of patients with newly diagnosed PAH and CTEPH, diagnosed in one of the reference or competence centers of the French PAH network. | From enrollement to the end of study at day 1 |
| Measure | Description | Time Frame |
|---|---|---|
| Compare broad screening of hair and urine for environmental toxicants and drugs in PAH and CTEPH patients | From enrollement to the end of study at day 1 | |
| Compare each of the 4 exposure domains (toxic agents (drugs and medications), occupational exposures, environmental exposures and socioeconomic factors) individually between PAH and CTEPH patients. |
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Inclusion Criteria:
Consent signed by the participant
Age ≥ 18 years
Free subject, under no legal protection
Good understanding of the French language, allowing to answer the questionnaires
Incident patients with pre-capillary PH confirmed by cardiac catheterization (PAPm > 20mmHg, PCP ≤ 15 mmHg, RVP > 2UW) having had the right diagnostic cardiac catheterization within one year (≤1 year) :
Exclusion Criteria:
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Major patients understanding French language with pre-capillary PH confirmed by cardiac catheterization during the year (PAH without associated pathology and CTEPH class 4)
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Etienne-Marie JUTANT, MD-PhD | Contact | +335 49 44 33 49 | etienne-marie.jutant@chu-poitiers.fr |
| Name | Affiliation | Role |
|---|---|---|
| Céline ABONNEAU, Project Manager | Poitiers University Hospital | Study Chair |
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| ID | Term |
|---|---|
| D000081029 | Pulmonary Arterial Hypertension |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
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| ID | Term |
|---|---|
| D011795 | Surveys and Questionnaires |
| D012494 | Sampling Studies |
| ID | Term |
|---|---|
| D003625 | Data Collection |
| D004812 | Epidemiologic Methods |
| D008919 | Investigative Techniques |
| D017531 | Health Care Evaluation Mechanisms |
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Two strands of hair (or beard or axillary hair) will be taken, along with a urine and blood sample.
|
| Samples | Other | Two strands of hair (or beard or axillary hair) will be taken, along with a urine and blood sample. |
|
| From enrollement to the end of study at day 1 |
| Compare the exposome of PAH patients and CTEPH patients, divided into 4 domains including toxic agents using a combination of direct measurements on hair and urine and indirect measurements via questionnaires. | Compare the exposome of PAH patients and CTEPH patients, divided into 4 domains including toxic agents (drugs and medications), occupational and environmental exposures and socioeconomic factors, using a combination of direct measurements on hair and urine and indirect measurements via questionnaires. | From enrollement to the end of study at day 1 |
| D002318 |
| Cardiovascular Diseases |
| D011787 | Quality of Health Care |
| D017530 | Health Care Quality, Access, and Evaluation |
| D011634 | Public Health |
| D004778 | Environment and Public Health |
| D016020 | Epidemiologic Study Characteristics |