Table. 1—

Randomised controlled trials of pulmonary arterial hypertension pharmacotherapies in chronic thromboembolic pulmonary hypertension(CTEPH)

Target pathwayProstacyclinNitric oxideEndothelin
First author [Ref.]Olschewski [19]Suntharalingam [20]Jais [21]
Subjects n57 CTEPH (203 total)#19 CTEPH157 CTEPH
Duration of study12 weeks12 weeks16 weeks
Primary end-point6MWD + NYHA functional class6MWD6MWD or PVR
Primary end-point metYes (total study population)NoNo: 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.