PT - JOURNAL ARTICLE AU - Tyler Pitre AU - Johnny Su AU - Sonya Cui AU - Ryan Scanlan AU - Christopher Chiang AU - Renata Husnudinov AU - Muhammad Faran Khalid AU - Nadia Khan AU - Gareth Leung AU - David Mikhail AU - Pakeezah Saadat AU - Shaneela Shahid AU - Jasmine Mah AU - Lisa Mielniczuk AU - Dena Zeraatkar AU - Sanjay Mehta TI - Medications for the treatment of pulmonary arterial hypertension: a systematic review and network meta-analysis AID - 10.1183/16000617.0036-2022 DP - 2022 Sep 30 TA - European Respiratory Review PG - 220036 VI - 31 IP - 165 4099 - http://err.ersjournals.com/content/31/165/220036.short 4100 - http://err.ersjournals.com/content/31/165/220036.full SO - EUROPEAN RESPIRATORY REVIEW2022 Sep 30; 31 AB - Background: There is no consensus on the most effective treatments of pulmonary arterial hypertension (PAH). Our objective was to compare effects of medications for PAH.Methods: We searched MEDLINE, Embase, the Cochrane Central Register of Controlled Trials and Clinicaltrials.gov from inception to December 2021. We performed a frequentist random-effects network meta-analysis on all included trials. We rated the certainty of the evidence using the Grades of Recommendation, Assessment, Development, and Evaluation approach.Results: We included 53 randomised controlled trials with 10 670 patients. Combination therapy with endothelin receptor antagonist (ERA) plus phosphodiesterase-5 inhibitors (PDE5i) reduced clinical worsening (120.7 fewer events per 1000, 95% CI 136.8–93.4 fewer; high certainty) and was superior to either ERA or PDE5i alone, both of which reduced clinical worsening, as did riociguat monotherapy (all high certainty). PDE5i (24.9 fewer deaths per 1000, 95% CI 35.2 fewer to 2.1 more); intravenous/subcutaneous prostanoids (18.3 fewer deaths per 1000, 95% CI 28.6 fewer deaths to 0) and riociguat (29.1 fewer deaths per 1000, 95% CI 38.6 fewer to 8.7 more) probably reduce mortality as compared to placebo (all moderate certainty). Combination therapy with ERA+PDE5i (49.9 m, 95% CI 25.9–73.8 m) and riociguat (49.5 m, 95% CI 17.3–81.7 m) probably increase 6-min walk distance as compared to placebo (moderate certainty).Conclusion: Current PAH treatments improve clinically important outcomes, although the degree and certainty of benefit vary between treatments.Combination therapy with PDE5i plus ERA and riociguat monotherapy were most effective at reducing clinical worsening and improving 6MWD in PAH. https://bit.ly/3xlZqDF