Pulmonary Artery Remodelling With Bosentan

• Status: Completed
• Trial end date: 2010-06-01
• Target enrollment: 0
• Participant gender: All

Summary

The main purpose of this study is to investigate whether bosentan (Tracleer®) affects the wall thickness of the pulmonary arteries in patients with idiopathic pulmonary arterial hypertension (iPAH) and PAH related to systemic sclerosis (PAH-SSc).

The second purpose is to investigate if bosentan affects the enlargement of small vessels in the lungs in response to natural chemicals in patients with iPAH and PAH-SSc.

• Phase: Phase 4
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Actelion
• Treatments: Bosentan

Criteria

Inclusion Criteria : · Men or women >18 years of age.·

• Symptomatic (modified NYHA class III) iPAH or PAH-SSc·

• PAH confirmed by right heart catheterization performed within 3 months before enrolment mPAP > 25 mmHg, PCWP < 15 mmHg and PVR > 3 mmHg/l/min.

• Women of childbearing potential must have a negative pre-treatment pregnancy test and use a reliable method of contraception during study treatment and for 3 months after study treatment termination.

• Bosentan naïve patients

Exclusion Criteria : · PAH other than iPAH or PAH-SSc

• Significant vasoreactivity during right heart catheterization defined as a fall in mPAP to < 40 mmHg with a decrease >= 10 mmHg and with a normal cardiac index (>= 2.5 l/min.m2)· Severe obstructive lung disease: FEV1/FVC < 0.5

• Severe restrictive lung disease: TLC < 0.7 of normal predicted value

• Hemoglobin <75% of the lower limit of the normal range· Systolic blood pressure < 85 mmHg

• Body weight < 40 kg

• Pregnancy or breast-feeding

• Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C.

• Baseline aminotransferases, i.e., aspartate aminotransferases (AST) and/or alanine aminotransferases (ALT) > 3 times the upper limit of the normal (ULN) range.

• Treatment for iPAH or PAH-SSc within 1 month before start of study treatment, excluding warfarin and acute administration of vasodilators for vascular reactivity testing during heart catheterization.

• Treatment with epoprostenol or other prostacyclin analogs for iPAH or PAH-SSc within 1 month before start of study treatment

• Treatment with glibenclamide (glyburide), fluconazole ketoconazole or ritonavir within 1 week before start of study treatment.

• Current treatment with cyclosporine A or tacrolimus

• Hypersensitivity to bosentan or any of the excipients of its formulation.

• Patient who received an investigational drug (such as sildenafil) within 3 months before start of study treatment

• Conditions that prevent compliance with the protocol or adherence to therapy.