Chronic Thromboembolic Pulmonary Hypertension (CTEPH), Pulmonary Arterial Hypertension (PAH)
Conditions
Keywords
Pulmonary hypertension, Exposome
Brief summary
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.
Interventions
OTHERQuestionnaires
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).
OTHERSamples
Two strands of hair (or beard or axillary hair) will be taken, along with a urine and blood sample.
Sponsors
Poitiers University Hospital
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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 * 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) : * Cases: Patients with PAH without associated pathology: idiopathic or heritable, or with features of venous/capillary involvement, according to the clinical classification of PH * Controls: Patients with chronic thromboembolic PH (CTEPH, class 4), according to the clinical classification of PH.
Exclusion criteria
* 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 * Patients benefiting from enhanced protection, i.e. minors, persons deprived of their liberty by a judicial or administrative decision, persons staying in a health or social establishment, adults under legal protection, and patients in emergency situations
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 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 | From enrollement to the end of study at day 1 |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of outdoor and indoor environmental exposures | From enrollement to the end of study at day 1 | 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 | From enrollement to the end of study at day 1 | 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 | From enrollement to the end of study at day 1 | 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 | From enrollement to the end of study at day 1 | 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. |
Countries
France
Contacts
CONTACTEtienne-Marie JUTANT, MD-PhD
CONTACTCéline ABONNEAU, Project manager
Outcome results
None listed