Pulmonary arterial hypertension (PAH) is commonly associated with connective tissue disorders (CTDs). This study provides a contemporary assessment of the economic burden of CTD + PAH and PAH in the United States. Eligible adult patients identified from Optum's deidentified Clinformatics® Data Mart Database (10/01/2015‐09/30/2021) were classified into mutually exclusive cohorts based on recorded diagnoses: (1) CTD + PAH, (2) PAH, (3) CTD, (4) control without CTD/PAH. The index date was a randomly selected diagnosis date for PAH (CTD + PAH, PAH cohorts) or CTD (CTD cohort), or a random date (control cohort). Entropy balancing was used to balance characteristics across cohorts. Healthcare costs and healthcare resource utilization (HRU) per patient per month (PPPM) were assessed for ≤12 months postindex and compared among balanced cohorts. A total of 552,900 patients were included (CTD + PAH:
n
= 1876; PAH:
n
= 8177; CTD:
n
= 209,156; control:
n
= 333,691). Average total all‐cause costs were higher for CTD + PAH than PAH cohort ($16,854 vs. $15,686 PPPM;
p
= 0.02); both cohorts incurred higher costs than CTD and control cohorts ($4476 and $2170 PPPM; all
p
< 0.001). Average HRU PPPM was similar between CTD + PAH and PAH cohorts (inpatient stay: 0.15 vs. 0.15, outpatient visits: 4.23 vs. 4.11; all
p
> 0.05), while CTD and control cohorts incurred less HRU (inpatient stay: 0.07 and 0.03, outpatient visits: 2.67 and 1.69; all
p
< 0.001). CTD + PAH and PAH are associated with a substantial economic burden. The incremental burden attributable to PAH versus the general population and patients with CTD without PAH highlights significant unmet needs among PAH patients.