Background <p>The implementation of targeted drug therapy results in a significant improvement in both survival rates and quality of life among patients diagnosed with pulmonary arterial hypertension (PAH), concurrently imposing a greater financial burden on them. The use of pharmacoeconomic evaluation based on decision-analytical models is extensively employed in the rational allocation of healthcare resources.</p> Objectives <p>The present study conducted a systematic review of the literature on the pharmacoeconomic evaluation of drugs for treating PAH, with a focus on summarizing the composition and sources of parameters in decision-analytical models. This study aims to provide methodological guidance for future economic research.</p> Methods <p>The review was conducted across six databases (PubMed, Embase, the Cochrane Library, CNKI, VIP, WanFang Data) and two health technology assessment agency websites (NHS EED, INAHTA). The characteristics of each study and the compositional details of the decision-analytical models are extracted.</p> Results <p>In total, 13 published studies were included. The pharmacoeconomic evaluation methods employed in the studies included cost-effectiveness analysis (CEA) and cost-utility analysis (CUA). The decision analysis models employed in all 13 studies were Markov models. The models were all constructed on the basis of the World Health Organization (WHO) functional class, with variations in parameter settings and sources.</p> Conclusions <p>All 13 Markov models provided useful insight into PAH modeling. Future research in this field can employ these research methods according to diverse research objectives. The utility values were derived from a single source; therefore, future studies should evaluate the quality of life in patients with PAH across varying disease severities.</p>