For more information about these clinical trials please contact the Cardiomyopathy Treatment Program at 319-356-1028, or by mail at University of Iowa Hospitals and Clinics, 200 Hawkins Dr., T416 General Hospital, Iowa City, IA 52242.

• Vision
• FREEDOM - C
• FREEDOM - M
• Compass II
• Monotherapy on PAH
• Registries

Vision

"A Randomized, Double-blind, Placebo-Controlled Study to Evaluate the Safety and Efficacy of the Addition of Inhaled Iloprost in Patients with Pulmonary Arterial Hypertension (PAH) Receiving Oral Sildenafil."

• Study looks at the safety and efficacy of 2 FDA indicated medications for PAH taken simutaneously (sildenafil and iloprost).
• Iloprost is an inhaled synthetic prostacyclin. Sildenafil is a phosphodiesterase Type 5 Inhibitor.
• Requries current use of sildenafil at a stale dose for the indication of PAH.
• Randomized to either 6 inhalations of illoprost, placebo or combination of iloprost and placebo inhalations.
• Study participation includes 16 weeks of blinded study drug followed by 32 weeks of open-label iloprost.
• Primary investigator is James Carroll, MD
• Primary coordinator is Holly Verry, RN

This study is an international, multi-center, randomized, double-blind, placebo-controlled study in subjects with PAH who are currently receiving approved therapy for their PAH (i.e., endothelin receptor antagonist and/or phosphodiesterase-5 inhibitor). Study visits will occur at 4 week intervals for 16 weeks with the key measure of efficacy being the 6-minute walk test. Study procedures include routine blood tests, medical history, physical exams, disease evaluation, and exercise tests. One optional substudy is also a part of FREEDOM-C at select centers - a hemodynamic substudy with a right heart catheterization at Baseline and Week 16.

Patients who complete all assessments for 16-weeks will also be eligible to enter a 36 month open-label, extension phase study (FREEDOM - EXT).

Inclusion Criteria:

• Between 12 and 65 years of age, inclusive.
• Body weight at least 45 kg (approximately 100 pounds)
• PAH that is either idiopathic/familial; associated with repaired congenital systemic-to-pulmonary shunts (repaired > 5 years); associated with Collagen Vascular Disease; associated with HIV
• Currently receiving an approved endothelin receptor antagonist and/or an approved phosphodiesterase-5 inhibitor for at least 90 days and on a stable dose for at least the last 30 days
• Previous testing (e.g., right heart catheterization, echocardiography) consistent with the diagnosis of PAH.
• Reliable and cooperative with protocol requirements.
• The primary investigator is James Carroll, MD
• The study coordinator is Cyndi Larew, RN

This study is an international, multi-center, randomized (2:1 active:placebo), double-blind, placebo-controlled study in subjects with PAH who are NOT currently receiving approved therapy for their PAH. Study visits will occur at 4 week intervals for 12 weeks (with an additional visit at week 11) with the key measure of efficacy being the 6-minute walk test. Study procedures include routine blood tests, medical history, physical exams, disease evaluation, and exercise tests. Two optional substudies are also a part of FREEDOM-M at select centers - a hemodynamic substudy with a right heart catheterization at Baseline and Week 12 and a genetics and biomarkers substudy with blood samples collected at Baseline and Week 12.

Patients who complete all assessments for 12-weeks will also be eligible to enter a 36 month open-label, extension phase study (FREEDOM - EXT).

Inclusion Criteria:

• Between 12 and 65 years of age, inclusive.
• Body weight at least 45 kg (approximately 100 pounds)
• PAH that is either idiopathic/familial; associated with repaired congenital systemic-to-pulmonary shunts (repaired > 5 years); associated with Collagen Vascular Disease; associated with HIV
• Previous testing (e.g., right heart catheterization, echocardiography) consistent with the diagnosis of PAH.
• Reliable and cooperative with protocol requirements
• The primary investigator is James Carroll, MD
• The study coordinator is Cyndi Larew, RN

Effects of Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Morbidity and Mortality in Symptomatic Patients With Pulmonary Arterial Hypertension - A Multicenter, Double-Blind, Randomized, Placebo-Controlled, Parallel Group, Prospective, Event Driven Phase IV Study.

Inclusion Criteria:

1.  Males or females equal or greater than 12 years of age=
(Women of childbearing potential must have a negative pre-treatment pregnancy test and must use reliable methods of contraception.)

2.  Patients with symptomatic PAH

3.  Patients with the following types of PAH belonging to WHO Group I:

1. Idiopathic (IPAH)
2. Familial (FPAH)
3. Associated with (APAH):
   1. Collagen vascular disease with normal left ventricular function (ejection fraction (EF) > 50%)
   2. Congenital systemic-to-pulmonary shunts iii.Drugs and toxins

4.  PAH diagnosed by right heart catheter within 2 years prior to screening showing:

• Mean pulmonary arterial pressure (mPAP) equal to or greater than 25 mmHg AND
• Pulmonary capillary wedge pressure (PCWP)equal to or smaller than 15 mmHg

5.  Treatment with a stable dose of sildenafil equal to or greater than 20 mg t.i.d. for at least 12 weeks prior to randomization (no sildenafil dosage adjustment should occur in this period) 7)6MWT between 150m and 420m

This study will investigate the effects of the combination of bosentan and sildenafil. This approach appears as a highly attractive therapeutic option to address the multiple pathophysiological mechanisms that are present in PAH. Several advantages are obvious: both substances are orally available and both are usually well tolerated. In addition, bosentan and sildenafil have different mechanisms of action, aiming at different intracellular targets. This implies that combination therapy is likely to result in synergistic effects. It will be the first event-driven study in PAH ever conducted.The dual treatment with bosentan and sildenafil might further prevent or reverse vasoconstriction, thrombosis and changes in vascular structure, thus ultimately improving the prognosis of PAH patients.

Involvement is the study lasts days.

The primary investigator is James Carroll, MD

The study coordinator is Page Scovel, RN

We are currently involved in two Industry sponsored registries for pulmonary hypertension. These registries track patient histories, symptoms, medications and testing related to pulmonary artery hypertension. This information will be used to evaluate participant treatments and outcomes over longer periods of time.

The primary investigator is James Carroll, MD

The study coordinators are Page Scovel, RN, Holly Verry, RN, and Cyndi Larew, RN