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European Respiratory Review logoLink to European Respiratory Review
. 2017 Jan 3;26(143):155067. doi: 10.1183/16000617.6067-2015

“Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension.” Irene M. Lang, Sean P. Gaine. Eur Respir Rev 2015; 24: 630–641.

PMCID: PMC10901166  PMID: 28049123

Unfortunately there was an error in the entry for selexipag in table 2 of this article. Instead of “none” for background therapy it should have stated “‘none, ERA, PDE-5i or both’”. Please find the corrected table below.

TABLE 2.

Key randomised controlled clinical trials of drugs that target the prostacyclin pathway

First author [ref.] Year (trial acronym) Background therapy Drug Patients n Duration Primary end-point Primary end-point met?
Epoprostenol #
 Rubin [37] 1990 None i.v. epoprostenol 24 8 weeks Change in total pulmonary resistance Yes
 Barst [38] 1996 None i.v. epoprostenol 81 12 weeks Change in 6MWD Yes
 Badesch [39] 2000 None i.v. epoprostenol 111 12 weeks Change in 6MWD Yes
 Badesch[40] 2009 None i.v. epoprostenol 102 3 years Survival No
 Humbert[41] 2004 (BREATHE-2) None i.v. epoprostenol with bosentan or placebo 33 16 weeks Change in total pulmonary resistance No
 Simonneau [42] 2008 (PACES) i.v. epoprostenol Sildenafil or placebo 267 16 weeks Change in 6MWD Yes
Iloprost
 Olschewski [43] 2002 None Inhaled iloprost or placebo 203 12 weeks Composite ≥10% increase in 6MWD and improvement in WHO FC Yes
 Hoeper [44] 2006 (COMBI) Bosentan Inhaled iloprost 40 12 weeks Change in 6MWD No
 McLaughlin [45] 2006 Bosentan Inhaled iloprost 67 12 weeks Change in 6MWD and WHO FC Yes
Treprostinil +
 Simonneau [46] 2002 None s.c. treprostinil or placebo 470 12 weeks Change in 6MWD Yes
 Jing [47] 2013 (FREEDOM-M) None Oral treprostinil or placebo 349 12 weeks Change in 6MWD Yes
 Tapson [48] 2012 (FREEDOM-C) ERA, PDE-5i or both Oral treprostinil or placebo 350 16 weeks Change in 6MWD No
 Tapson [49] 2013 (FREEDOM-C2) ERA, PDE-5i or both Oral treprostinil or placebo 310 16 weeks Change in 6MWD No
 McLaughlin [50] 2010 (TRIUMPH-I) Bosentan or sildenafil Inhaled treprostinil or placebo 235 12 weeks Change in 6MWD 10–60 min after inhalation Yes
Beraprost §
 GaliÉ [51] 2002 (ALPHABET) None Oral beraprost or placebo 130 12 weeks Change in 6MWD Yes
 Barst [52] 2003 None Oral beraprost or placebo 116 12 months Difference in disease progression Yes
Selexipag
 McLaughlin [53] 2015 (GRIPHON) None, ERA, PDE-5i or both Oral selexipagƒ 1156 3 years Time to first morbidity or mortality event Yes

6MWD: 6-min walking distance; WHO: World Health Organization; FC: functional class; ERA: endothelin receptor agonist; PDE-5i: phosphodiesterase type 5 inhibitor. #: approved for continuous i.v. administration for pulmonary arterial hypertension (PAH) WHO FC III–IV by the US Food and Drug Administration (FDA) in 1995; : approved for aerosol administration for PAH WHO FC III in the European Union and Australia in 2003, and PAH WHO FC III–IV by the FDA in 2004; +: approved for s.c. administration for PAH WHO FC II–IV by the FDA and Health Canada in 2002; §: approved for oral administration for idiopathic PAH in Japan in 1995 [36]; ƒ: not approved at time of publication.


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