CLINICAL TRIALS

Pulmonary hypertension is an active area with respect to clinical trials of new pharmacological agents. Only the initial trial of intravenous epoprostenol has used survival as an outcome measure. Following this study surrogate markers of outcome especially six minute walk distance have been used. Most of the currently active trials involve combinations of the different disease targeted therapies or allow the possibility of combination therapy. The major exception to this are patients with Dana Point Group II - IV pulmonary hypertension (i.e. not pulmonary arterial hypertension) where monotherapy is not yet proven.

Randomised Controlled Trials of Disease Targeted Treatment in Progress in Adults

a) IPAH & APAH

Sponsor	Trial Name	Intervention	Inclusion Criteria	Other Disease Targeted Treatment Allowed	Duration	Target for Recruit- ment
Georgetown University		Bosentan	SSc with exercise induced PH	No	16 weeks	40
Actelion	COMPASS 2	Bosentan added to Sildenafil	IPAH, FPAH, APAH, on sildenafil for minimum of 12 weeks	No	16 weeks	250
Bayer	PATENT 2	Riociguat	IPAH	Yes	12 weeks + extension	462
United Therapeutics	ATPAHSS	Tadalafil and/or ambrisentan	Systemic Sclerosis with pulmonary hypertension, WHO II or III	No	36 weeks	63
Novartis Pharma-ceuticals	IMPRES	Imatinib	IPAH, WHO II to IV	Yes	24 weeks	200
Virginia Common-wealth University		Carvedilol	IPAH or assoc PAH, WHO II to IV	Yes	6 months	24
Pfizer		Bosentan +/- sildenafil	PAH	Yes	12 weeks	106
University of Heidelberg Still recruiting		The influence of respiratory and exercise therapy on oxygen uptake, quality of life and right ventricular function	PAH	Yes	15 weeks	90
University of Heidelberg Still recruiting		The influence of respiratory and exercise therapy on 6 minute walk distance and quality of life	PAH	Yes	16 weeks	34
Hospital de Clinicas de Porto Alegre Still recruiting		Inspiratory muscle training	PAH	Yes	8 weeks	34
University of Heidelberg Still recruiting		Exercise training	PAH	Yes	16 weeks	34
Hannover Medical School Still recruiting		3 week In-patient rehabilitation programme	PAH	Yes	3 weeks	100
Seoul National University Still recruiting	ULTIMATE	Udenafil	Mild Pulmonary Hypertension	Yes	12 weeks	40
Golden Jubilee National Hospital Still recruiting		Apelin Infusion	IPAH	Yes	1 day (exploratory study)	63
The Cleveland Clinic Ongoing but not recruiting		Oral iron supplementation	IPAH plus iron defficiency	Yes	12 weeks	25
Actelion Still recruiting	GRIPHON	Selexipag	PAH	Yes	6 months plus extended study period; long term extension follows on	670
Hillel Yaffe Medical Centre Unknown		Normatec? pump	Right heart failure and leg oedema	Yes	5 days	20
University of Alberta Still recruiting First to Humans, Phase I study. Imperial College also particpating		Dichloroacetate (DCA)	IPAH, FPAH, APAH	Yes	16 weeks	40
Rabin Medical Centre Not yet recruiting		Vardenafil	PAH	Iloprost	14 weeks	20
Stanford University Unknown		Tadalafil, Tyvaso; in combination or stand alone therapy	IPAH	No	48 weeks	30
Stanford University Still recruiting		Tacrolimus (effect on BMPRII signalling)	IPAH, FPAH, CTDPAH, CHDPAH, APAH, porto pulmonary PH	Yes, but bosentan excluded	16 weeks	40
National Institutes of Health Clinical Centre Still recruiting		Spironolactone (effect on inflammation and blood vessel function)	PAH	Yes	24 weeks	70
GSK Still recruiting	AMBITION	Tadalafil, ambrisentan; in combination or stand alone therapy	PAH	No	24 weeks plus extended timeline	545
University of Pennsylvania Still recruiting		Anastrolzole (phase II, effect on TAPSE)	PAH	Yes	12 weeks	18
Imperial College London Still recruiting		Ferinject?(Iron injections) (phase II)	IPAH	Yes	12 weeks	60
University of Heidelberg Still recruiting		Exercise and Respiratory Therapy	Connective Tissue related Pulmonary Hypertension	Yes	15 weeks	45
National Institute of Allergy and Infectious Diseases Still recruiting		Rituximab (Phase II)	Systemic sclerosis pulmonary hypertension	No	48 weeks	80
Medical University of Gratz Still recruiting		Ambrisentan	Porto-pulmonary Hypertnesion	No	24 weeks	24
Tufts Medical Centre Still recruiting		Ambrisentan	Porto-pulmonary Hypertnesion	No	24 weeks	30
University of Calafornia Still recruiting		Iloprost	Congenital Heart Disease PAH	Yes	12 weeks	16

= SPVU is taking part.

b) Non-IPAH studies

Sponsor	Trial Name	Intervention	Inclusion Criteria	Other Disease Targeted Treatment Allowed	Duration	Target for Recruit- ment
Royal Brompton & Harefield NHS Foundation Trust	B-PHIT	Bosentan	IPF or NSIP with pulmonary hypertension	No	16 weeks + extension	48
The University of North Carolina		Warfarin	Sickle Cell Disease with pulmonary hypertension	No	12 months	20
Capital Medical University		Simvastatin	CTEPH or PH secondary to congenital heart disease with PASP > 50mmHg
University Teaching Hospital Hall in Tirol	BADDHY	Bosentan	Diastolic heart failure with pulmonary hypertension, PCWP > 15mmHg, NYHA II to III	No	24 weeks	60
Hospital General Universitario Greorio Mara?on	SIOVAC	Sildenafil	Previous heart valve intervention with pulmonary hypertension	No	6 months	354
Bayer	CHEST 2	Riociguat	CTEPH		16 weeks + extension	270
University of Cincinnati	BOSAPAH	Bosentan	Sarcoidosis with pulmonary hypertension, NYHA II to III	No	48 weeks	60
University of Heidelberg Still recruiting		The influence of respiratory and exercise therapy on 6 minute walk distance and quality of life	CTEPH post endarte- rectomy	Yes	15 weeks	35
Italian Association of Hospital Pneumonologists Still recruiting	SPHERIC-1	Sildenafil	COPD with pulmonary hypertension	No	16 weeks	32
Italian Association of Hospital Pneumonologists Still recruiting	TAPIT-1	Inhaled Treprostinil	COPD with pulmonary hypertension	No	16 weeks	32
National Institutes of Health Clinical Centre Still recruiting		Education plus an exercise programme	PAH associated with ILD	Yes	10 weeks	175
University Hospital, Bordeaux Still recruiting	DHEA-HTAP	Dehydroepian-drosterone (DHEA)	COPD with pulmonary hypertension	No	52 weeks	60
University of North Carolina Unknown	SAPH	Tadalafil (open label)	Sarcoidosis associated PAH	No	20 weeks	20
Medical Research Network GmbH Active, not recruiting	CTREPH	Sub cutaneous treprostinil (Open label)	Inoperable CTEPH	Yes	24 weeks	unknown

= SPVU is taking part.

SPVU STUDIES