Epidemiology

Pulmonary arterial hypertension (PAH) is a rare but probably underestimated condition.

No published data on the estimated prevalence of PH in developing countries is available. However, the prevalence of PH is thought to be much higher than reported in developing countries where diseases that may promote pulmonary vascular disease (schistosomiasis, sickle cell disease, human immunodeficiency virus infection, liver cirrhosis and congenital heart disease) are relatively common [4-6].

In Europe, the prevalence of PAH has been reported at 15-60 subjects per million population with an annual incidence of 5-10 cases per million per year.

PAH affects women more commonly than men, and patients of all ages may develop the disease. The mean age at diagnosis has been reported at 36 to 50 years, with more recent series and series including PAH related to other systemic diseases (particularly scleroderma) reporting older mean ages at diagnosis.[20]