Randomised controlled trial data on the use of medical therapies in patients with chronic thromboembolic pulmonary hypertension

CHEST-1 [68]Suntharalingam et al. [69]MERIT-1 [70]BENEFiT [71]
Patients n2611980157
Active treatmentRiociguatSildenafilMacitentanBosentan
Treatment duration weeks16122416
Population n (%)
 Inoperable189 (72.4)10 (52.6)80 (100)96 (70.1)
 Persistent/recurrent PH post-pulmonary endarterectomy72 (27.6)9 (47.4)041 (29.9)
Use of PAH therapy at baseline n (%)
 No261 (100.0)19 (100.0)31 (38.8)157 (100.0)
 Yes0049 (61.3)0
Primary end-pointChange in 6MWD from baseline to week 16Change in 6MWD from baseline to week 12PVR at week 16 as a percentage of baselineChange in PVR and 6MWD from baseline to week 16 (co-primary end-points)
Treatment effect on 6MWD+46 (25–67) m, p<0.001#+18 (−24–59) m, p=ns+34 (3–65) m, p<0.05#+2 (−23–27) m, p=ns
Treatment effect on PVR−246 (−303– −190) dyn·s·cm−5, p<0.001#−197 (−389– −6) dyn·s·cm−5, p<0.050.84 (0.70–0.99), p<0.05+−24%§ (−32– −16%), p<0.0001+
Treatment effect on mPAP−5 (−7–−3) mmHg, p<0.001#−6 (−12–0) mmHg, p=ns−2 (−6–2) mmHg, p=ns#−3 (−5–0) mmHg, p=ns

Data are presented as n, n (%) or mean difference (95% CI), with p-value, between active and control groups, unless otherwise stated. PH: pulmonary hypertension; PAH: pulmonary arterial hypertension; 6MWD: 6-min walk distance; PVR: pulmonary vascular resistance; mPAP: mean pulmonary arterial pressure; ns: nonsignificant. #: treatment effect is the least-squares mean difference; : corresponds to a 16% reduction, PVR decreased by 206 dyn·s·cm−5 in the macitentan group and 86 dyn·s·cm−5 in the placebo group; +: treatment effect is the ratio of geometric means; §: PVR decreased by 146 dyn·s·cm−5 in the bosentan group and increased by 30 dyn·s·cm−5 in the placebo group.