TABLE 2.

Chronic thromboembolic pulmonary hypertension (CTEPH)-specific randomised controlled trials of medical therapies that include quality of life (QoL) measurement

Trial name (acronym) [ref.]	Treatment	Trial design	Number/type of patients	Primary end-point	Primary end-point met?	QoL/HRQoL tools used	QoL/HRQoL results	Correlation with other end-points
Bosentan effects in inoperable forms of CTEPH (BENEFiT) [45]	Bosentan 62.5 mg twice daily up to 125 mg twice daily	Multicentre, randomised, double-blind, placebo-controlled	157/CTEPH	Change in PVR or 6MWD after 16 weeks of treatment compared with placebo	PVR end-point met, but not 6MWD	SF-36	No significant difference between bosentan and placebo groups	None performed
Long-term use of sildenafil in inoperable CTEPH [58]	Sildenafil 40 mg three times daily	Randomised, double-blind, placebo-controlled	19/CTEPH	Change in 6MWD after 12 weeks of treatment compared with placebo	No	CAMPHOR	Significant improvement in the activity component	None performed
CTEPH soluble guanylate cyclase- stimulator trial (CHEST-1) [51]	Riociguat 2.5 mg three times daily	Multicentre, randomised, double-blind, placebo-controlled	261/CTEPH	Change in 6MWD after 16 weeks of treatment compared with placebo	Yes	EQ-5D, LPH	Significant improvement compared with baseline and compared with placebo in EQ-5D Change in LPH score did not reach level of significance	6MWD at baseline and 16 weeks, change in 6MWD at baseline and 16 weeks for EQ-5D and LPH

HRQoL: health-related quality of life; PVR: pulmonary vascular resistance; 6MWD: 6-min walking distance; SF-36: 36-item Short-Form Health Survey; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; EQ-5D: EuroQol-5D; LPH: Living with Pulmonary Hypertension.