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.
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
Patients classified as HTP groups 2, 3 or 5
Patients with a diagnosis of PAH associated with a connective tissue disease, HIV, portal hypertension, congenital heart disease, bilharzia, or a drug or toxic cause considered certain according to the international classification (Aminorex, Benfluorex, Carfilzomib, Dasatinib, Dexfenfluramine, Fenfluramine, Methamphetamines, Mitomycin C, adulterated rapeseed oil)
Patients with signs of right heart failure requiring amines and/or intensive care hospitalization at time of visit (may be included at a later visit)
Patients refusing hair sampling or having no hair > 2 cm to allow sampling
Major patients understanding French language with pre-capillary PH confirmed by cardiac catheterization during the year (PAH without associated pathology and CTEPH class 4)
Case-control
Prospective
Questionnaires
Other interventionPatients 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).
Samples
Other interventionTwo strands of hair (or beard or axillary hair) will be taken, along with a urine and blood sample.
To compare the exposome assessed based on the concentration of 190 environmental toxins, drugs and medications of interest in the hair and urine of patients newly diagnosed with PAH or CTEPH
To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of outdoor and indoor environmental exposures
Compare indoor and outdoor exposure questionnaires as well as the EPICES socioeconomic assessment questionnaire. Compare environmental exposures using a composite score of indoor and outdoor pollution. Compare concentrations of NO₂, PM10, and PM2.5 to pollutants assessed based on patients' addresses. Compare broad-based screening of hair and urine for the presence of environmental toxins.
To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of occupational exposures
Compare the results of work schedules based on standardized questionnaires. Compare the results of job-exposure matrices applied to work schedules.
To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of medications exposures
Compare the medication history corresponding to a 2-year exposure assessment for each drug studied.
To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of drug exposures
Compare substance use questionnaires. Compare questionnaires on substance use disorders. Compare questionnaires on associated risky behaviors. Compare broad-range drug screening of hair and urine.
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