The epidemiology of Pulmonary Hypertension with Lung Disease and/or Hypoxia (PH-LD), including PH with Interstitial Lung Disease (PH-ILD), remains unclear in France. This retrospective longitudinal study aims to characterize the prevalence and incidence of PH-LD/ILD from the French hospital claims database (PMSI).

In the absence of ICD-10 codes to identify PH-LD and the sub-group PH-ILD patients, an algorithm was adapted with input from six European PH experts. Patients with recorded PH from Jan 2017-Dec 2019, and LD up to 24 months prior to or 60 days after the PH index date were included. Those with other PH Group records were excluded from the baseline. The ?narrow? group included patients who received a right heart catheterization. The ?broad? group included patients with both LD and left heart disease (typically associated with Group 2 PH). Prevalence and incidence are expressed per million people.

Both PH-LD/ILD patients had a mean age of 67 (SD, 13), 51% of PH-LD and 61% of PH-ILD were women.  The estimated prevalence of PH-LD was 42 (95%CI, 40-44) with an incidence of 28 (95%CI, 26-31). The narrow and broad PH-LD prevalence was 26 (95%CI, 24-28) and 52 (95%CI, 50-54), respectively. The prevalence of PH-ILD was 17 (95%CI, 14-0.19), and the incidence 12 (95%CI, 9.0-14).

While healthcare interactions occurring outside the hospital are not captured in the PMSI database, even applying a broader definition, the prevalence of the PH-LD and PH-ILD is rare in France.