Pulmonary arterial hypertension (PAH) is a progressive disease with no cure. Healthcare resource utilization (HCRU; hospitalization, outpatient visits, and drug utilization) before diagnosis and productivity loss (sick leave and disability pension) before and after PAH diagnosis are not well known. By linking several Swedish national databases, this study have estimated the societal costs in a national PAH cohort (
n
= 749, diagnosed with PAH in 2008−2019) 5 years before and 5 years after diagnosis and compared to an age, sex, and geographically matched control group (
n
= 3745, 1:5 match). HCRU and productivity loss were estimated per patient per year.
The PAH group had significantly higher HCRU and productivity loss compared to the control group starting already 3 and 5 years before diagnosis, respectively. HCRU peaked the year after diagnosis in the PAH group with hospitalizations (mean ± standard deviation; 2.0 ± 0.1 vs. 0.2 ± 0.0), outpatient visits (5.3 ± 0.3 vs. 0.9 ± 0.1), and days on sick leave (130 ± 10 vs. 13 ± 1) significantly higher compared to controls. Total costs during the entire 10‐year period were six times higher for the PAH group than the control group. In the 5 years before diagnosis the higher costs were driven by productivity loss (76%) and hospitalizations (15%), while the 5 years after diagnosis the main cost drivers were drugs (63%), hospitalizations (16%), and productivity loss (16%). In conclusion, PAH was associated with large societal costs due to high HCRU and productivity loss, starting several years before diagnosis. The economic and clinical burden of PAH suggests that strategies for earlier diagnosis and more effective treatments are warranted.