Epidemiology

Pulmonary arterial hypertension (PAH) is a rare disease, with an estimated prevalence of 48-55 cases per million adults and an annual incidence of 2.4-6.0 per million people.[4][5]​​​ Several registries have been established to collect data on baseline characteristics and outcomes of patients with PAH, with the first set up in the 1980s by the US National Institutes of Health.[6] Idiopathic pulmonary arterial hypertension (IPAH) is the most common type of PAH reported in the registries, accounting for 30% to 50% of cases.[7] The age and sex distribution of the disease appears to have evolved over time. The mean age of patients in the first US registry was 36 years, with a female-to-male ratio of 1.7:1.[6] Contemporary registries from France and the US (REVEAL) later described a mean age at diagnosis of 50 years.[8][9]​​​​​​​ A significant proportion of patients (9% in the French registry) were older than 70 years.[8] While the French registry confirmed a female-to-male ratio of 1.6:1, the US registry depicted a much higher female preponderance, with a female-to-male ratio of 3.9:1.[8][9]​​​ Another registry of six European countries found that the median age of IPAH diagnosis was 71 years and that younger patients had a female-to-male ratio of 2.3:1 while the female-to-male ratio in older adult patients was 1.2:1.[10] In the UK and Ireland, registry data from 2001 to 2009 showed an increase in the average age of IPAH diagnosis, from 45 to 52 years.[11] Comorbidities including systemic hypertension, obesity, type 2 diabetes mellitus, and ischemic heart disease are common in patients with IPAH.

[Figure caption and citation for the preceding image starts]: Clinical classification and prevalence of pulmonary hypertension. CTEPH, chronic thromboembolic pulmonary hypertension; CpCPH, combined post- and pre-capillary pulmonary hypertension; IpcPH, isolated post-capillary pulmonary hypertension; mPAP, mean pulmonary arterial pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance.Adapted from Eur Heart J, Volume 43, Issue 38, 7 October 2022, 3618–731; used with permission [Citation ends].com.bmj.content.model.Caption@6a653bf2

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