Randomised controlled trials of pulmonary arterial hypertension pharmacotherapies in chronic thromboembolic pulmonary hypertension(CTEPH)
| Iloprost | Sildenafil | Bosentan | |
| Target pathway | Prostacyclin | Nitric oxide | Endothelin |
| First author [Ref.] | Olschewski [19] | Suntharalingam [20] | Jais [21] |
| Subjects n | 57 CTEPH (203 total)# | 19 CTEPH | 157 CTEPH |
| Duration of study | 12 weeks | 12 weeks | 16 weeks |
| Primary end-point | 6MWD + NYHA functional class | 6MWD | 6MWD or PVR¶ |
| Primary end-point met | Yes (total study population) | No | No: 6MWD |
| Yes: PVR | |||
| Change in PVR versus placebo dyn·s·cm−5 | -105* (pre-inhalation)-335§ (post-inhalation) | -197* | -176* |
| Change in 6MWD versus placebo m | +36.4* (PPH = 58.8; nPPH = 12) | +17.5 | +2.2 |
PVR: pulmonary vascular resistance; 6MWD: 6-min walking distance; NYHA: New York Heart Association; PPH: primary pulmonary hypertension; nPPH: nonPPH (including CTEPH and pulmonary hypertension associated with anorexigen use or scleroderma). #: separate analyses for the CTEPH subgroup were not presented but type of pulmonary hypertension had no significant effect on the response to treatment; ¶: independent co-primary end-points; §: significance versus placebo not assessed. *: p<0.05.