RT Journal Article SR Electronic T1 Medications for the treatment of pulmonary arterial hypertension: a systematic review and network meta-analysis JF European Respiratory Review JO EUROPEAN RESPIRATORY REVIEW FD European Respiratory Society SP 220036 DO 10.1183/16000617.0036-2022 VO 31 IS 165 A1 Pitre, Tyler A1 Su, Johnny A1 Cui, Sonya A1 Scanlan, Ryan A1 Chiang, Christopher A1 Husnudinov, Renata A1 Khalid, Muhammad Faran A1 Khan, Nadia A1 Leung, Gareth A1 Mikhail, David A1 Saadat, Pakeezah A1 Shahid, Shaneela A1 Mah, Jasmine A1 Mielniczuk, Lisa A1 Zeraatkar, Dena A1 Mehta, Sanjay YR 2022 UL http://err.ersjournals.com/content/31/165/220036.abstract 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