OBJECTIVES : Pulmonary Arterial Hypertension (PAH) is a rare, debilitating and potentially fatal disease. This study aims to quantify the economic and social burden of PAH in Spain.

METHODS : A systematic literature review was conducted to collect direct and indirect costs associated with incident and prevalent patients. Average annual costs per patient were estimated by multiplying the consumption of each resource by its unit cost, differentiating the functional class (FC) of the patient. Total annual costs in Spain, per FC, were also calculated, taking into account the ranges of prevalence and incidence of the disease in 2020. An expert committee (one cardiologist, one pneumologist, one pharmacist and one nurse) validated the information on resource consumption and provided primary information on pharmacological consumption. Unit costs were approximated using official tariffs and salaries in Spain. The study was conducted from a societal perspective.

RESULTS : The annual number of PAH patients in Spain was considered to be between 770 and 1,157 (in CF I-II 31.8%; CF III 61.3%; CF IV 6.9%), of which 145 were diagnosed in 2020. The average annual total cost was estimated at €96,911 per patient (CF I-II: €63,667; CF III: €100,368; CF IV: €218,023), being €40,272 for incident patients (CF I-II: €24,540; CF III: €42,371; CF IV: €94,130). The total annual cost of PAH in Spain was estimated at between €66,420,290 and €103,914,738 depending on the prevalence considered. Direct health care costs accounted for 63% of the total cost, followed by indirect costs (25%) and direct non-health care costs (12%).

CONCLUSIONS : PAH places a considerable economic and social burden on patients and their families, the healthcare system and society as a whole. Efforts must be made to improve the health and management of these patients, especially those with poorer functional status.