Pulmonary Artery Remodelling With Bosentan

Actelion Pharmaceuticals

Status and phase

Completed

Phase 4

Conditions

Hypertension, Pulmonary

Treatments

Drug: bosentan

Study type

Interventional

Funder types

Industry

Identifiers

NCT00595049

AC-052-416

Details and patient eligibility

About

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.

Enrollment

11 patients

Sex

All

Ages

18+ years old

Volunteers

No Healthy Volunteers

Inclusion and exclusion 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.