Table 1: General characteristics of the included studies.

ReferencesYear published, countryPerspectiveModel typeTarget populationTreatmentComparatorCost componentsTime horizonDiscount rate (%)Source of effectiveness and safety data

Highland et al. [12]2003, USAUnclearMarkov modelPatients with PAHBosentanEpoprostenol, treprostinilDrug, diluent, per diem, hospitalization, home health, Hickman catheter, liver functionOne yearNAThree studies
Garin et al. [13]2009, USAUnclearMarkov modelPatients with FC III and IV PAHBosentanEpoprostenol, treprostinil, iloprost, sitaxentan, ambrisentan, sildenafilDrug, per diem, pain medications, hospitalization/clinic visit, intravenous line infections, laboratory testsOne yearNATwo RCTs
Coyle et al. [14]2016, CanadaHealthcare systemMarkov modelPatients with FC II and III PAHBosentanAmbrisentan, sildenafil, tadalafil, supportive careDrugs, monitoring/therapeutic procedures (includes liver function tests, pregnancy test, echocardiograms, renal function, and blood work), hospital/ER/clinic visits (includes general practitioner visits, specialist visits, nurse visits, hospitalizations, emergency room visits, therapeutic procedures), Supportive care drugsLifetime5A network meta-analysis
Dranitsaris et al. [15]2009, CanadaCanadian healthcare systemCost-minimization analysis (CMA)Patients with FC II and III PAHBosentanAmbrisentan, sitaxentan, sildenafilDrug acquisition, medical consultations and visits, laboratory and diagnostic procedures, functional studies, other healthcare-related resources, alternative pharmacotherapy3 years3Nine placebo-controlled trials
Wlodarczyk et al. [16]2006, AustraliaA healthcare payer perspectiveAn excel modelPatients with iPAHBosentanConventional therapyExercise test, lung function, chest x-ray, echocardiogram, electrocardiogram, blood tests, specialist, total medical15years5Two aforementioned pivotal clinical trials and their long-term open-label extensions
Stevenson et al. [17]2009, UKNational Health ServiceMarkov modelPatients with iPAH or PAH-CTD of FC IIIBosentanPalliative therapyDrug acquisition, home delivery, palliative careLifetime3.5Two RCTs
Fan et al. [18]2016, ChinaUnclearMarkov modelPatients with PAHBosentanPalliative therapyDrugs, monitoring/therapeutic proceduresLifetime3.5Patient registration and follow-up data for charity project
Barbieri et al. [19]2014, ItalyNational Health SystemAn excel modelPatients with FC II and III PAHBosentanAmbrisentanDrug acquisition cost, direct medical costs (includes visits to professionals, laboratory tests, concomitant medications, hospitalizations)3 yearsUnclearTwo separate double-blind studies

Note. PAH: pulmonary arterial hypertension; FC: functional class; NA: not applicable; RCT: randomized controlled trial; CTD: connective tissue disease; CMA: cost minimization analysis.