What is the Role of the Exposome in Pulmonary Hypertension

Обзор

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.

Вмешательства

• Другое Questionnaires
  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).
• Другое Samples
  Two strands of hair (or beard or axillary hair) will be taken, along with a urine and blood sample.

Первичные конечные точки

• 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 [Срок оценки: From enrollement to the end of study at day 1]

Вторичные конечные точки (4)

• To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of outdoor and indoor environmental exposures [Срок оценки: From enrollement to the end of study at day 1]
• To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of occupational exposures [Срок оценки: From enrollement to the end of study at day 1]
• To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of medications exposures [Срок оценки: From enrollement to the end of study at day 1]
• To compare the exposome of patients newly diagnosed with PAH or CTEPH in terms of drug exposures [Срок оценки: From enrollement to the end of study at day 1]

Критерии участия

Критерии включения

• 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.

Критерии исключения

• 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

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Случай-контроль

Центры проведения

Франция · 10 центров

• Bordeaux University Hospital — Bordeaux
• Brest University Hospital — Brest
• Le Kremlin Bicetre Hospital — Le Kremlin-Bicêtre
• Lille University Hospital — Lille
• Louis Pradel Hospital — Lyon
• Nancy University Hospital — Nancy
• Poitiers University Hospital — Poitiers
• Saint Etienne University Hospital — Saint-Etienne
• … и ещё 2 центра

Идентификаторы

NCT: NCT07172334 · 2025-A00986-43

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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