Sponsor	Trial name	Intervention	Inclusion criteria	Other disease targeted treatment allowed	Duration	Target for recruitment
IPAH and APAH
CoTherix	VISION	Inhaled iloprost added to sildenafil	IPAH	Yes	16 weeks + extension	180
Lung Rx	TRIUMPH 1	Inhaled treprostinil	IPAH, CTDPH, HIV (WHO class III & IV)	Yes	12 weeks + extension	150*
United Therapeutics	FREEDOM-M	Oral treprostinil	IPAH, CTDPH, HIV, repaired CHD	Yes	12 weeks + extension	150
United Therapeutics	FREEDOM-C	Oral treprostinil	IPAH, CTD-APAH, HIV, repaired GUCH	Yes	16 weeks + extension	300
Actelion	COMPASS-2	Bosentan added to sildenafil	IPAH, CTDPH, CHD – on sildenafil	Yes	16 weeks + extension	600
Pfizer	A1481243	Sildenafil added to bosentan	PAH on bosentan	Yes	12 weeks + extension	106
Pfizer	A1481244	Low dose sildenafil	IPAH, CTD-APAH, repaired GUCH	No	12 weeks	174
Lilly ICOS	PHIRST-1	Tadalafil	IPAH, CTDPH, ASD, repaired CHD	Yes	16 weeks + extension	400*
Imperial College London/MRC	SIPHT	Simvastatin	IPAH, CTDPH (WHO class II & III)	Yes	12 months	60
University Hospital, Giesen	SIPHT	Aspirin, simvastatin	IPAH, CTDPH		6 months + extension	40
Assistance Publique – Hopitaux de Paris		Escitalopram	IPAH, CTDPH, HIV, repaired CHD (WHO class II & III)	Yes	16 weeks	30
Northern Therapeutics Inc	PHACeT	Endothelial progenitor cells with the eNOS gene	IPAH		3 months	18^†
Novartis	CST1571E2203	Imatinib	IPAH, CTDPH (WHOII- IV)	Yes	6 months + extension	60
Zhejiang University		Autologous endothelial progenitor cells	IPAH			40
University of Heidelberg	STOP-POAH-001	Atorvastatin	IPAH, CTD-APAH, repaired GUCH	Yes	12 weeks	150
University of Heidelberg		Exercise	CTD-APAH	Yes	15 weeks
Georgetown University/Actelion	IRB 06-043	Bosentan	SSc-APAH (WH01-11)	No	16 weeks	40
National Heart, Lung, and Blood Institute	07-H-0177	Sildenafil	Sickle cell disease, APAH	No	16 weeks + extension	300

Non-PAH studies
EPIX Pharmaceuticals		5-HT2B antagonist (PRX-08066)	COPD with pulmonary hypertension	No		72
National Heart, Lung, and Blood Institute		Sildenafil	IPF with pulmonary hypertension	No	2 weeks	20
Herlev Hospital		Taldalafil	COPD with PH	No	4 weeks	20
University of Heidelberg		Exercise	CTEPH after PEA	Yes	15 weeks	30
Actelion		Tezosentan	Patients undergoing cardiopulmonary bypass with mean PAP >30 mm Hg	No	24 h	270

*Recruitment recently closed; †non-randomised.