Figures & data
Table S1 General characteristics of the included studies
RubinLJMendozaJHoodMTreatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trialAnn Intern Med19901124854912107780 OlschewskiHSimonneauGGalieNInhaled iloprost for severe pulmonary hypertensionN Engl J Med200234732232912151469 GalieNHumbertMVachieryJLEffects of beraprost sodium, an oral prostacyclin analogue, in patients with pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled trialJ Am Coll Cardiol2002391496150211985913 GalieNOlschewskiHOudizRJAmbrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2Circulation20081173010301918506008 BadeschDBTapsonVFMcGoonMDContinuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trialAnn Intern Med200013242543410733441 BarstRJRubinLJLongWAA comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertensionN Engl J Med19963342963018532025 BarstRJMcGoonMMcLaughlinVBeraprost therapy for pulmonary arterial hypertensionJ Am Coll Cardiol2003412119212512821234 RubinLJBadeschDBBarstRJBosentan therapy for pulmonary arterial hypertensionN Engl J Med200234689690311907289 HumbertMBarstRJRobbinsIMCombination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2Eur Respir J20042435335915358690 GalieNBeghettiMGatzoulisMABosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled studyCirculation2006114485416801459 ChannickRNSimonneauGSitbonOEffects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled studyLancet20013581119112311597664 GalieNRubinLHoeperMTreatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trialLancet20083712093210018572079 JingZCYuZXShenJYVardenafil in pulmonary arterial hypertension: a randomized, double-blind, placebo-controlled studyAm J Respir Crit Care Med20111831723172921471085 TapsonVFTorresFKermeenFOral treprostinil for the treatment of pulmonary arterial hypertension in patients on background endothelin receptor antagonist and/or phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C study): a randomized controlled trialChest20121421383139022628490 TapsonVFJingZCXuKFOral treprostinil for the treatment of pulmonary arterial hypertension in patients receiving background endothelin receptor antagonist and phosphodiesterase type 5 inhibitor therapy (the FREEDOM-C2 study): a randomized controlled trialChest201314495295823669822 JingZCParikhKPulidoTEfficacy and safety of oral treprostinil monotherapy for the treatment of pulmonary arterial hypertension: a randomized, controlled trialCirculation201312762463323307827 HoeperMMLeuchteHHalankMCombining inhaled iloprost with bosentan in patients with idiopathic pulmonary arterial hypertensionEur Respir J20062869169417012628 McLaughlinVVGaineSPBarstRJEfficacy and safety of treprostinil: an epoprostenol analog for primary pulmonary hypertensionJ Cardiovasc Pharmacol20034129329912548091 McLaughlinVVOudizRJFrostARandomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertensionAm J Respir Crit Care Med20061741257126316946127 GhofraniHAGalieNGrimmingerFRiociguat for the treatment of pulmonary arterial hypertensionN Engl J Med201336933034023883378 BarstRJOudizRJBeardsworthATadalafil monotherapy and as add-on to background bosentan in patients with pulmonary arterial hypertensionJ Heart Lung Transplant20113063264321256048 WilkinsMRPaulGAStrangeJWSildenafil versus Endothelin Receptor Antagonist for Pulmonary Hypertension (SERAPH) studyAm J Respir Crit Care Med20051711292129715750042 PulidoTAdzerikhoIChannickRNMacitentan and morbidity and mortality in pulmonary arterial hypertensionN Engl J Med201336980981823984728 SimonneauGBarstRJGalieNContinuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trialAm J Respir Crit Care Med200216580080411897647 SimonneauGRubinLJGalieNAddition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trialAnn Intern Med200814952153018936500 GalieNGhofraniHATorbickiASildenafil citrate therapy for pulmonary arterial hypertensionN Engl J Med20053532148215716291984 McLaughlinVVBenzaRLRubinLJAddition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trialJ Am Coll Cardiol2010551915192220430262 HiremathJThanikachalamSParikhKExercise improvement and plasma biomarker changes with intravenous treprostinil therapy for pulmonary arterial hypertension: a placebo-controlled trialJ Heart Lung Transplant20102913714920022264 GalieNBarberaJAFrostAEInitial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial HypertensionN Engl J Med201537383484426308684 ZhuangYJiangBGaoHZhaoWRandomized study of adding tadalafil to existing ambrisentan in pulmonary arterial hypertensionHypertens Res20143750751224646647 GalieNMullerKScaliseAVGrunigEPATENT PLUS: a blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertensionEur Respir J2015451314132225657022 McLaughlinVChannickRNGhofraniHABosentan added to sildenafil therapy in patients with pulmonary arterial hypertensionEur Respir J20154640541326113687