OBJECTIVES: The aim of this study was to identify cost drivers in Pulmonary Arterial Hypertension (PAH) in the US through a systematic literature review of economic and humanistic burden.

METHODS: A systematic literature review was conducted adhering to methodology published by the Cochrane Collaboration and the National Institute for Health and Clinical Excellence (NICE) in the UK. Population, Intervention & Comparators, Outcomes and Study Design (PICOS) criteria were used to identify resource utilization and economic burden studies in PAH published from credible databases (EMBASE, MEDLINE, MEDLINE In-Process) over the last 10 years.

RESULTS: A total of 170 publications were extracted with most publications from the US. Total all-cause costs per patient per month (PPPM) in the US (all costs reported in 2019 $USD) were found to range from $2,494 to $11,037, driven primarily by medical costs. Medical costs accounted for a larger portion of total costs than pharmacy costs within both all-cause ($2,113 to $7,625 PPPM) and PAH-related ($293 to $5,086 PPPM) health care costs; among all-cause medical cost components, inpatient admission costs ($1,115 to $2,146 PPPM) contributed the highest costs, followed by outpatient costs ($954 to $1,944 PPPM). Although total costs increased after initiation of PAH therapies, increased pharmacy costs were partially offset by reduced medical costs ($1,506 to $57,409 decreased annual medical costs from baseline).

CONCLUSIONS: Management of PAH carries a considerable economic burden with the major cost drivers consisting of primarily medical costs. All-cause costs vary widely in current literature, which may be due to heterogeneity in study population and cost components.