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Rare Disease

Spotlight Poster Session C

(SLC-036) Characteristics and mortality of patients with Pulmonary Arterial Hypertension in France: A 2010-2023 Nationwide Study from the French Health Data System (SNDS)

Tuesday, August 26, 2025
12:15 PM - 1:15 PM ET

    Presenting Author(s)

  • CN

    Charifa Nazoiri, PharmD

    EPI-PHARE, French National Agency for Medicines and Health Products Safety (ANSM)
    Saint-Denis, Ile-de-France, France

    • Co-Author(s)

  • AW

    Alain Weill, PhD, MD

    EPI-PHARE (French National Agency for Medicines and Health Products Safety (ANSM) and French National Health Insurance (CNAM)), Saint-Denis, France, France

  • AB

    Antoine Beauvais, MD

    Service de pneumologie et de soins intensifs pneumologiques - Centre de référence de l'Hypertension pulmonaire
    Kremlin-Bicêtre, France

  • MZ

    Mahmoud Zureik, PhD, MD

    Professor in UVSQ Paris Saclay University, Head / Director of Epi Phare Laboratory Gis ANSM / CNAM
    EPI-PHARE Scientific Interest Group in Epidemiology of Health Products from the French National Agency for the Safety of Medicines and Health Products and the French National Health Insurance, St Denis, France ; Paris Saclay University, Université Versailles Saint Quentin (UVSQ - EDSP)
    ST DENIS, France

  • JB

    Jeremie Botton, PhD,PharmD

    EPI-PHARE, France

  • NH

    Nadia Haddy, PhD, MD

    EPI-PHARE, French National Agency for Medicine and Health Product Safety (ANSM) and the French National Health Insurance Center (CNAM)
    ST DENIS, France

Background: Pulmonary arterial hypertension (PAH) is a rare and severe disease which has been associated to the use of several drugs (e.g.,benfluorex, aminorex). In addition to the registries, the French Health Data System (SNDS) can serve as a useful tool for studying this condition.

Objectives: To describe the characteristics of patients with PAH between 2013 and 2023, and assess all-cause mortality.

Methods: We conducted a cohort study using SNDS database to identify adults under 70 years with a PAH.
PAH patients were identified by a hospitalization with an ICD-10 code I27.0 or I27.2 and at least two dispensations of a PAH-specific treatment within 6 months before or after a right heart catheterization. We assessed comorbidities in the 5 years prior to the first hospitalization (index date). Overall and sex-specific mortality were evaluated using Standardized Mortality Ratio (SMR), comparing mortality of patients with PAH to that of the general population via Poisson regression models. The Kaplan-Meier estimator was used to estimate median survival time.

Results: We identified 3722 PAH patients of whom 46.8% were women. Median age was 61.0 years (interquartile range [IQR] 52.0-66.0), with women were younger than men (median age 59.0 years vs. 63.0 years). There was a social gradient, with 14.4% belonging to quintile of the least deprived to 24 % belonging to the quintile of the most deprived. The most prevalent comorbidities were chronic lung disease (54.4%) and coronary heart disease (22.0%). Median overall survival was 6.7 years and longer in women (8.4 years vs. 4.5 years in men). Compared to the general population, patients with PAH had a mortality risk 12.5 times higher (SMR: 12.5 [95% CI: 11.9, 13.1]). Women had a higher SMR (15.6 [95% CI: 14.4–17.0] vs. 11.1 [95% CI: 10.4, 11.8]).

Conclusions: This large national study highlights that PAH patients represent a high-risk population with comorbidities and a higher mortality compared to the general population, with gender difference. Our findings underscore the potential of the SNDS database to conduct PAH drug-induced studies.