Table 3– Efficacy parameters

Characteristics	Baseline		3 weeks				15 weeks
	Mean±sd	95% CI	Mean±sd	Median	95% CI	p-value	Mean±sd	Median	95% CI	p-value
Subjects n		183			183				103
6MWD by PH aetiology m
All	425±106	410–441	493±110		478–510	<0.001	506±104		486–528	<0.001
IPAH/HPAH (n=87/87/53)	448±89	429–467	523±89		503–542	<0.001	543±74		522–563	<0.001
APAH (n=46/46/18)	427±113	393–461	482±123		445–518	<0.001	454±132		392–516	<0.001
CTEPH (n=31/31/18)	417±110	377–457	482±126		436–528	<0.001	512±81		473–551	0.001
PH (n=19/19/11)	327±100	279–375	403±86		362–444	<0.001	407±118		328–486	<0.001
Difference from baseline 6MWD by PH aetiology m
All			68±46		61–75	<0.001	78±49.5		68–88	<0.001
IPAH/HPAH (n=87/53)			75±43		69–92	<0.001	85±48.9		71–98	<0.001
APAH (n=46/18)			54±44		36–80	<0.001	56±48.8		32–80	<0.001
CTEPH (n=31/18)			65±48		52–100	<0.001	85±49.8		60–110	0.001
PH (n=19/11)			76±55		44–118	<0.001	72±48		41–105	<0.001
6MWD in WHO-FC subgroups m
WHO-FC II (n=26/26/13)	507±100	466–548	572±100		532–613	<0.001	588±57		554–623	<0.001
WHO-FC III (n=137/137/76)	424±86	410–439	494±93		478–510	<0.001	511±85		492–530	<0.001
WHO-FC IV (n=18/18/10)	239±95	190–291	298±90		253–342	<0.001	287±108		209–364	0.010
Difference from baseline 6MWD in WHO-FC subgroups m
All (n=183/103)			68±46		61–75	<0.001	78±50		68–88	<0.001
WHO-FC II (n=26/13)			65±41		49–82	<0.001	85±53		53–117	<0.001
WHO-FC III (n=137/76)			70±46		62–78	<0.001	79±48		68–90	<0.001
WHO-FC IV (n=18/10)			59±51		33–84	<0.001	63±61		19–107	0.010
Improvement in 6MWD in WHO-FC subgroups %
WHO-FC II (n=26/13)			14.3±11.1	12.9	9.8–18.7	<0.001	18.5±14.1	16.1	10.0–27.0	<0.001
WHO-FC III (n=137/76)			17.5±13.5	14.7	15.2–20.0	<0.001	19.3±13.3	14.6	16.3–22.4	<0.001
WHO-FC IV (n=18/10)			25.0±27.0	17.4	11.5–38.4	<0.001	26.3±30.1	17.8	4.8–47.8	0.010
Cardiopulmonary exercise testing
Peak V′_O₂ per kg mL·min⁻¹·kg⁻¹	12.2±3.5		13.6±4			<0.001	13.9±3.8			<0.001
Peak V′_O₂ mL·min⁻¹	907±276		1006±308			<0.001	1011±292			<0.001
Eq_CO₂ at AT	45.2±9.6		43.6±10.6			0.249	44.3±10.8			0.225
V′_O₂ at AT mL·min⁻¹	692±214		712±236			0.216	790±261			0.007
Oxygen pulse	7.5±2.1		7.9±2.4			<0.001	7.7±2.4			0.001
PASP rest mmHg	59±20		57±20			0.028	56±20			0.016
PASP maximum mmHg	91±26		91±26			0.542	95±26			0.512
Heart rate rest min⁻¹	77±13		74±11			<0.001	80±13			0.009
Heart rate maximum min⁻¹	122±19		127±22			<0.001	133±20			<0.001
RRsys rest mmHg	117±17		114±15			0.016	113±14			0.019
RRdia rest mmHg	78±14		75±9			<0.001	77±9			0.128
RRsys maximum mmHg	150±26		151±24			0.615	150±24			0.719
RRdia maximum mmHg	86±14		86±12			0.617	87±12			0.667
Borg scale	15.6±2		15.6±2			0.896	15.7±2			0.100
Workload maximum W	64±24		77±28			<0.001	80±27			<0.001

6MWD: 6-min walk distance; IPAH: idiopathic pulmonary arterial hypertension; HPAH: heritable pulmonary arterial hypertension; APAH: associated pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; PH: pulmonary hypertension; WHO: World Health Organization; FC: functional class; V′_O₂: oxygen uptake; Eq_CO₂: respiratory equivalence for carbon dioxide; AT: anaerobic threshold; PASP: systolic pulmonary artery pressure; RRsys: systolic systemic blood pressure; RRdia: diastolic systemic blood pressure. p-values are the same for absolute values and differences in 6MWD; Kruskall Wallis test showed no significance in intergroup comparison, p=0.446 (3 weeks) and p=0.993 (15 weeks). p-values for 6MWD from two-sided t-test, for cardiopulmonary exercise testing from Wilcoxon test.